International Journal of Surgical Pathology RSS feed -- OnlineFirst Articles

Alveolar Soft-Part Sarcoma of Paranasal Sinuses

Dezanzo, P., Lifschitz Mercer, B., Czernobilsky, B., Rosai, J. — Thu, 19 Nov 2009 00:33:56 PST

Metanephric Stromal Tumor Arising in a Patient With the Neurofibromatosis Type 1 Syndrome

McDonald, O. G., Rodriguez, R., Bergner, A., Argani, P. — Wed, 28 Oct 2009 22:25:44 PDT

Metanephric stromal tumor (MST) is a recently recognized benign renal stromal tumor. MST is thought to be part of a spectrum of benign metanephric renal lesions, which also includes the epithelial lesion metanephric adenoma and the mixed stromal–epithelial lesion metanephric adenofibroma. Metanephric lesions may represent hyperdifferentiated counterparts to Wilms’ tumor (WT). MST characteristically shows renovascular angiodysplasia and juxtaglomerular (JG) cell hyperplasia. This is remarkably similar to the renal pathology described in neurofibromatosis-1 (NF-1) syndrome, a condition which is also associated with WT. Here, we report the first case of MST arising in a patient with NF-1. The patient presented with hypertension, and the MST was associated with florid angiodysplasia and JG cell hyperplasia. This case tightens the link between NF-1, WT, and MST.

Intrafollicular Collagenous Crystalloids

Kacerovska, D., Michal, M., Kazakov, D. V. — Wed, 14 Oct 2009 04:40:50 PDT

The authors report an unusual case of intrafollicular collagenous crystalloids in an 86-year-old woman. The presence of collagenous crystalloids within the follicular epithelium is intriguing and has not been described previously.

For Valentine's Day: Adnexal Induction Over Dermatofibroma

Kacerovska, D., Michal, M., Kazakov, D. V. — Wed, 14 Oct 2009 04:40:51 PDT

Will the Modification of the Gleason Grading System Affect the Urology Practice?

Wed, 30 Sep 2009 05:49:44 PDT

The aim of the present study was to determine how the modified Gleason grading (mGG) system affects the score discrepancy between needle biopsy (NB) and radical prostatectomy (RP) and to investigate the effect of the modified scores on nomogram predictions. When the conventional Gleason grading (cGG) and mGG systems were compared, a new Gleason score was obtained in the NBs for 40 out of 97 patients (41.2%; P < .001) and in the RP specimens for 15 out of 97 patients (15.5%; P = .005). The agreement between the NBs and RP specimens rose from 31.9% to 44.3% with the mGG system (P = .017). However, when the predictions calculated with the location of modified Gleason scores in the Memorial Sloan Kettering Cancer Center nomogram were compared with those of the conventional Gleason scores, higher pathological stage and lower life expectancy predictions were obtained. Therefore, when a clinician is making a choice from therapeutic options, this change should be taken into account.

Tissue Microarray in Diffuse Large B-Cell Lymphomas: Are They Really Able to Identify Distinct Prognostic Groups in Lymphomas of Both Nodal and Extranodal Origin?

Wed, 30 Sep 2009 05:49:44 PDT

Aims. Diffuse large B-cell lymphomas (DLBCL) can be divided into different subgroups (germinal center B-cell-like [GCB] and non-GCB) according to their gene expression profiles. Immunohistochemistry has been proposed as a surrogate for identifying these subgroups, but data about its efficacy in providing prognostic information are conflicting. Methods and results. This study retrospectively analyzed a series of 105 DLBCL, defined as GCB and non-GCB according to CD10, bcl-6, and MUM1 expression. All patients received a first-line anthracycline-based (CHOP-like) chemotherapy. A total of 50 patients (48%) were identified as GCB and 55 (52%) as non-GCB. The overall response rate was 89% (94/105), with 62 (59%) complete response. Disease progressions were equally distributed between the 2 subgroups and were not significantly different (P = .756) considering the primary site of involvement (nodal or extranodal). The median follow-up was 62 months (range 5-126 months). Overall survival at 5 years was not significantly different between the groups (P = .3468) and was 72.3% and 66.6% for GCB and non-GCB, respectively. Conclusion. The results do not support the prognostic value of GCB and non-GCB immunohistochemical categories in DLBCL of both nodal and extranodal origin. Furthermore, a limited number of antigens may be not sufficient to identify the same patterns defined by cDNA microarray. Prospective studies are warranted to address this issue.

Cytogenetic Aberrations in Soft Tissue Tumors Harvested From Fresh Tissue Submitted for Surgical Pathology: A Single Institutional Experience

Matthews, A., Tang, M., Cooper, K. — Wed, 23 Sep 2009 01:48:16 PDT

Aims. Recurrent cytogenetic abnormalities have been reported in many types of soft tissue neoplasms, and the detection of these aberrations imparts diagnostic utility. The aim of this study is to demonstrate that classical karyotyping may be performed with minimal effort as an adjunct to surgical pathology on fresh tissue submitted for histopathological examination. To the authors’ knowledge, there are no recently published reviews in the literature in English of cytogenetic abnormalities in soft tissue tumors from a single institution.Methods. Conventional metaphase cytogenetics was performed in the authors’ cytogenetics laboratory on fresh tissue from mesenchymal tumors from their surgical pathology laboratory over a period of 4 years. Cytogenetics reports, clinical history, and histopathology were reviewed for 48 soft tissue tumors. Recurrent cytogenetic abnormalities were identified using the Mitelman Database of Chromosome Aberrations in Cancer and a review of the literature.Results. The authors reviewed 48 cases of benign and malignant soft tissue tumors, which included 28 distinct morphologies. Cytogenetic abnormalities were observed in 23 (48%) cases, a normal chromosome profile was found in 20 (42%) cases, and 5 (10%) cases had no karyotype reported because of specimen contamination or inadequacy. Of the 23 cases with an aberrant karyotype, 15 (65%) tumors had cytogenetic abnormalities present in 2 or more cases in the literature.Conclusions. Cytogenetic analysis demonstrated abnormal karyotypes in nearly half of this series of mesenchymal neoplasms, the majority of which consisted of recognized aberrations reported in the literature.

Pseudofungi: Coral Shapes and Bamboo Sticks in Lymph Node Sinuses

Singh, C., Manivel, J C., Pambuccian, S. E. — Thu, 10 Sep 2009 22:10:07 PDT

Renal Translocation Carcinoma With Expression of TFEB: Presentation of a Case With Distinctive Histological and Immunohistochemical Features

Sun, 16 Aug 2009 23:03:02 PDT

The authors describe a case of renal cell carcinoma with t(6; 11) (p21; q12) in a 22-year-old man. The tumor showed typical histological features of this neoplasm with 2 types of cells and hyaline nodules surrounded by small cells. Characteristically, the tumor showed cystic lumina with hyaline-papillary structures inside and in some areas large and irregular intratumoral vessels. On immunohistochemical study, the tumoral cells were positive for melanocytic markers and transcription factor EB, as also for AE1-AE3 and Cam5.2 anticytokeratin antibodies. The expression of epithelial markers in this neoplasm is uncommon, and we think it is an important finding because otherwise, if melanocytic markers such as HMB45 or Melan A are not used, some renal cell carcinomas with the t(6; 11) (p21; q12) may be mistaken for other more common renal cell carcinomas.

Vascular and Capsular Pseudoinvasion in Thyroid Neoplasms

Chebib, I., Opher, E., Richardson, M. E. — Mon, 10 Aug 2009 00:17:06 PDT

The diagnosis of follicular carcinoma of the thyroid requires the identification of capsular and/or vascular invasion, with the exception of distant metastases. However, several artifacts may mimic both vascular and capsular invasion. This article presents two cases of pseudo invasion in encapsulated follicular neoplasms. Their differentiation from true vascular and capsular invasion is discussed and the criteria for true invasion are reviewed.

Hodgkin's Lymphoma Presenting as Dominant Gastric Lesion in Immunocompetent Patients: Report of 5 Cases With EBV Analysis

Mon, 10 Aug 2009 00:17:08 PDT

Primary Hodgkin’s lymphoma (HL) of the stomach is an extremely rare entity. Most cases of gastric involvement by HL are observed in the setting of disseminated disease. The nonspecific nature of the symptoms and endoscopic findings, which include a large malignant-looking ulcer and mass or wall thickening, together with the considerable histological overlap between HLs and some non-HLs or undifferentiated carcinoma, make the surgical resection diagnosis extremely difficult. An accurate diagnosis is important as treatment and outcome differ significantly for these neoplasms. In small endoscopic gastric biopsies and even in postoperative specimens, the precise histological diagnosis of HL is particularly challenging. Here, the authors report 5 cases of 2 women and 3 men aged 22 to 68, with gastric involvement by classic HLs—3 primary gastric HLs and 2 as part of widespread disease. All 5 patients presented with digestive symptoms. At endoscopy, the lesions presented as ulcerated and elevated lesions, with or without mucosal thickening. Four patients were misdiagnosed in the preoperative biopsy or in the gastrectomy specimen. Association with Epstein-Barr virus (EBV) was detected in 4 cases, with a predominance of subtype A EBV. These cases illustrate the significant difficulties, both clinical and pathological, in achieving the diagnosis of HL involving the stomach in immunocompetent patients.

HER-2 Protein Overexpressed Breast Cancer Without Gene Amplification Shows Higher Hormone Receptor Expression Than HER-2 Protein Overexpressed Breast Cancer With Gene Amplification

Koo, J. S., Jung, W., Yang, W. I. — Mon, 10 Aug 2009 00:17:07 PDT

Cases of breast cancer showing human epidermal growth factor receptor-2 (HER-2) protein overexpression without corresponding gene amplification have been found in immunohistochemistry (IHC) and fluorescent in situ hybridization (FISH) results. We investigated the clinicopathological characteristics of cases showing HER-2 protein overexpression breast cancer without gene amplification (IHC3+/FISH–) and compared them to cases showing HER-2 protein overexpression breast cancer with gene amplification (IHC3+/ FISH+). This study was conducted on 90 patients with HER-2 IHC3+ breast cancer. The clinicopathological factors analyzed included tumor size, histological grade, nuclear grade, nodal involvement, and hormone receptor status. HER-2 IHC3+/FISH– breast cancer was found in 14 of 84 tumors (16.7%) and showed a statistically significant lower histological and nuclear grade (P = .000) and higher expression of estrogen receptors (ERs) and progesterone receptors (PRs) (P = .006) than IHC3+/FISH+ breast cancer. In conclusion, HER-2 IHC3+/FISH– breast cancer could be a subgroup showing lower histological/nuclear grade and higher expression of ERs/PRs.

Intravascular Papillary Endothelial Hyperplasia of the Renal Vein

Pelosi, G., Sonzogni, A., Viale, G. — Wed, 05 Aug 2009 23:02:33 PDT

Intravascular papillary endothelial hyperplasia (IPEH) is a benign, florid proliferation of endothelial cells forming hyaline papillae covered by monolayered cells, which has rarely been described in the renal vein, most often being equated to tumors because of the difficulties in correctly interpreting imaging findings and accordingly treated by nephrectomy. If the kidney is end-staged for preexisting medical diseases, the risk of overtreatment is obviously negligible, but overdiagnosing IPEH as an intravascular tumor, either benign or malignant, may be perilous for the patient because of the possible consequences on the clinical management.

Synchronous Follicular Lymphoma, Kaposi Sarcoma, and Castleman's Disease in a HIV-Negative Patient With EBV and HHV-8 Coinfection

Wed, 05 Aug 2009 23:02:33 PDT

The authors describe the case of a 65-year-old woman who was HIV negative and had a lymph node biopsy that showed concurrent follicular lymphoma (FL; grade 3A), Kaposi sarcoma (KS), and Castleman’s disease (CD) with coinfection by human herpes virus-8 (HHV-8) and Epstein-Barr virus (EBV). The lymphoma was positive for CD20, CD10, and BCL6 and negative for BCL2. Flow cytometry showed a clonal lambda B-cell population, and polymerase chain reaction (PCR) showed a clonal immunoglobulin heavy chain gene rearrangement, confirming a neoplastic B-cell process. Focally, the FL component showed numerous EBER1-positive cells, with rare HHV-8-positive cells. The KS component showed strong HHV-8 expression with rare EBER1-positive cells. The CD component showed scattered HHV-8, viral interleukin-6, and EBER1-positive cells. The simultaneous occurrence of a FL, KS, and CD in an HIV-negative patient expands the spectrum of HHV-8-positive neoplasms and suggests the possibility of HHV-8 rendering mature B-cells hyperresponsive to antigenic stimulation, providing an expanded target for second site mutations or cytokinedriven hyperplasia, culminating in lymphoma.

Acinar Cell Carcinoma With a Prominent Intraductal Growth Pattern: Case Report With Review of the Literature

Toll, A. D., Mitchell, D., Yeo, C. J., Hruban, R. H., Witkiewicz, A. K. — Tue, 07 Jul 2009 01:11:40 PDT

Acinar cell carcinoma of the pancreas is rare, accounting for less than 1% of carcinomas arising in the exocrine pancreas. Patients are typically between the fifth and seventh decade of life and show a 2:1 male predominance. Symptoms tend to be nonspecific, and approximately 50% of the patients have metastases at the time of presentation. There have been recent case reports of acinar cell carcinoma showing both intraductal and/or papillary patterns of growth that could potentially be mistaken for intraductal neoplasia. The cases reported up to date presented as solitary nodules. The authors describe the first case of acinar cell carcinoma with both intraductal and tubuloglandular growth diffusely involving the pancreas. In addition, the authors review the published cases reporting acinar cell carcinoma with intraductal and/or papillary growth patterns.

Elastofibromatous Changes in Tissues From Spinal Biopsies. A Degenerative Process Afflicting a Small but Important Subset Of Patients Operated for Spinal Canal Compression: Report of 18 Cases

Daum, O., Ferda, J., Curik, R., Choc, M., Mukensnabl, P., Michal, M. — Fri, 03 Jul 2009 00:28:44 PDT

Elastofibroma is a tumorlike lesion occurring usually in the subscapular region of elderly females. In this study, 18 cases of elastofibromatous tissue retrieved from the spinal canal were analyzed to elucidate its frequency and possible clinical associations. The patients included 8 men and 10 women with a mean age of 63.4 years (range, 40-84 years). The elastic nature of the material was confirmed by Weigert’s elastic stain and immunohistochemically. Elastofibroma was diagnosed in 6 patients, and elastofibroma-like tissue was found in 11 samples. The finding of elastofibromatous tissue was associated with clinical diagnosis of juxtaarticular cysts (3 cases), thickening of ligamentum flavum (4 cases), extradural expansion (2 cases), metastatic involvement of the dorsal spine (3 cases), and a vertebral fracture (1 case). Two cases were located above the transpedicular fixation. Elastofibromatous tissue may be found in approximately 2.6% of all spinal biopsies and it may take part in compression of spinal cord and nerve roots.

Skip Lesion of DIN (DCIS) in the Nipple in a Case of Breast Cancer

Tramm, T., Zuckerman, K., Tavassoli, F. A. — Fri, 03 Jul 2009 00:28:43 PDT

The authors present a case of breast cancer with a skip lesion of DIN 1 (ductal carcinoma in situ [DCIS] grade 1) in the nipple, leaving the base of the nipple free of disease. During the surgical procedure of nipple-sparing mastectomy (NSM) a frozen section evaluation of the base of the nipple and areola complex is often carried out to ascertain absence of any intraepithelial (in situ) or invasive neoplasms that could lead to nipple recurrences if left behind. This case illustrates a situation where a frozen section would have given a false-negative prediction of nipple involvement if the patient had been selected for NSM. This case illustrates the need to adhere to strict criteria for selecting patients eligible for NSM and to consider the use of additional preoperative and intraoperative measurements and methods to refine the selection of candidates for NSM and enhance the oncological safety of the procedure.

Comparison of Subtypes of Penile Squamous Cell Carcinoma From High and Low Incidence Geographical Regions

Chaux, A., Tamboli, P., Lezcano, C., Ro, J., Ayala, A., Cubilla, A. L. — Fri, 03 Jul 2009 00:28:44 PDT

There is a worldwide geographical variation in the incidence of penile squamous cell carcinoma (PSCC); some subtypes are HPV-related (warty, basaloid) while others (keratinizing variants) are not. The aims of this study were to compare the distribution of different histological subtypes of PSCC from regions of low (Paraguay, 144 cases) and high (USA, 157 cases) incidence and to identify and compare tumors with and without warty and/or basaloid morphology. The distribution of subtypes in the Paraguayan and the American series was: usual, 49.3 and 46.5%; verrucous, 8.3 and 7.6%; papillary NOS, 7.6 and 5.7%; warty, 6.9 and 8.3%; basaloid, 4.2 and 7.0%; sarcomatoid, 0.7 and 0.6%; adenosquamous, 3.5 and 0.6%; and mixed, 19.4 and 23.6%, respectively. The distribution of mixed PSCC was: warty-basaloid, 50.0 and 59.5%; usual-verrucous, 21.4 and 21.6%; usual-warty, 14.3 and 8.1%; usual-basaloid, 7.1 and 0.0%; usual-papillary, 3.6 and 5.4%; and others, 3.6 and 5.4%, respectively. In conclusion, we found no geographical difference in the incidence of histological subtypes (p = 0.6501), mixed PSCC (p = 0.5937) or HPV-related tumors (p = 0.2505). Geographical variation may be the result of staging variation at clinical presentation or of pathological diagnosis. The identification of similar histological subtypes in both series validates this classification approach for penile cancer. The tendency for typical SCC to mix with verrucous and papillary carcinomas and of the basaloid to preferentially mix with benign condyloma and condylomatous (warty) carcinomas would support the hypothesis of the existence of an etiologically different dual population of penile tumors.

High-Grade Astrocytomas Show Increased Nestin and Wilms's Tumor Gene (WT1) Protein Expression

Rushing, E. J., Sandberg, G. D., Horkayne-Szakaly, I. — Fri, 03 Jul 2009 00:28:44 PDT

Wilms’s tumor gene (WT1) is overexpressed in a variety of hematologic malignancies and solid tumors. Recently, WT1 protein has been considered as a molecular target of cancer immunotherapy for several solid tumors and as a tool for monitoring minimal residual disease in leukemia patients. There are only few investigations on WT1 expression in central nervous system neoplasms, which suggest that the WT1 gene may play an important role in tumorigenesis of primary astrocytic tumors and that high-grade tumors express high levels of WT1 proteins. We examined 50 low-grade and high-grade gliomas using tissue microarray and immunohistochemical methods to identify WT1 protein, P53, Ki-67, GFAP, NFP, EGFR, nestin, and Neu-N expression. WT1 and nestin shared overlapping expression in all gliomas and were increased in high-grade examples, highlighting their potential use as diagnostic and prognostic tumor markers. Our results support the combined role of WT1 and nestin in glial tumorigenesis and progression.

Pathologic and Clinical Features of Hodgkin Lymphoma-Like Posttransplant Lymphoproliferative Disease

Krishnamurthy, S., Hassan, A., Frater, J. L., Paessler, M., Kreisel, F. — Fri, 03 Jul 2009 00:28:43 PDT

Because of its rarity, pathologic and clinical features of Hodgkin lymphoma–like posttransplant lymphoproliferative disorder (HL-like PTLD) are not well understood, and it is unclear whether its biological behavior is more closely related to classical Hodgkin disease or to monomorphic B-cell PTLD. The authors compared 6 cases of HL-like PTLD with 5 cases of monomorphic B-cell PTLD for differences in histology, immunophenotype, and clinical behavior. Histologically, all cases of HL-like PTLD resembled classical HL with typical Reed–Sternberg (RS) cells and a cellular background mimicking mixed cellularity subtype. CD45 was absent on RS-like cells, but the expression pattern of B-cellassociated markers Oct-2 and BOB.1 resembled monomorphic B-cell PTLD. Whereas Epstein–Barr virus early RNA expression is normally restricted to RS cells of classical HL, it was expressed in both RS-like cells and background lymphocytes in HL-like PTLD. Although all patients diagnosed with monomorphic B-cell PTLD show no evidence of disease following treatment, half of the patients with HL-like PTLD relapsed or died, indicating a more aggressive clinical behavior. The findings suggest that HL-like PTLD represents a distinct clinicopathologic entity with an aggressive clinical course.

Lymphangiogenetic Characteristics of Triple Negativity in Node-Negative Breast Cancer

Liu, H.-T., Ma, R., Yang, Q.-F., Du, G., Zhang, C.-J. — Wed, 03 Jun 2009 02:48:14 PDT

Purpose. Triple-negativity breast cancer (TNBC), being negative for the estrogen receptor, the progesterone receptor, and the human epidermal growth factor receptor 2, represents a subgroup of breast cancer with a poor clinical outcome. The aim of the study was to determine whether TNBC is associated with lymphangiogenesis in node-negative breast carcinomas. Methods. The authors investigated the clinicopathologic characteristics, lymphatic vessel density (LVD), and expression of 2 lymphangiogenetic factors, vascular endothelial growth factor C (VEGF-C) and vascular endothelial growth factor D (VEGF-D), in 21 lymph node–negative TNBCs and 70 lymph node–negative non-TNBCs. Results. TNBC correlated with younger age (below 35 year) and higher histological grade. It also correlated with a higher intratumoral and peritumoral LVD, positive lymphatic invasion, and positive VEGF-C and VEGF-D expression. Conclusions. For the first time, this study indicated a link between triple-negativity breast cancer and lymphangiogenesis. Lymphangiogenesis might help explain the special malignant phonotype of breast cancer, and lymphangiogenesis inhibitors might be a novel choice for triple-negativity breast cancer patients.

Immunohistochemical Profile of Paratesticular Serous Papillary Adenocarcinoma and Tunica Vaginalis Facilitates Differentiation From Malignant Mesothelioma

Anchala, P. R., Dhir, R., Parwani, A. V., Zynger, D. L. — Wed, 03 Jun 2009 02:48:14 PDT

Testicular and paratesticular tumors resembling mullerian epithelium of the ovary are extremely uncommon. This study reports a case of paratesticular serous papillary adenocarcinoma (SPA) in an 87-year-old man that was misdiagnosed as malignant mesothelioma (MM) and is 37 years older than previous cases, highlighting that SPA does not occur exclusively in young patients as described. Immunohistochemistry revealed expression of pankeratin, CAM 5.2, CK7, CK903, BerEP4, vimentin, S100, and CEA and virtually no expression of CK5/6, CK20, calretinin, thrombomodulin, or glypican 3. Expression of adjacent nonneoplastic tunica vaginalis mesothelium was assessed in this patient and additional specimens. Profiles of paratesticular SPA and MM were summarized and compared with paratesticular mesothelium. Nontumoralstromal andentrapped mesothelial expression were 2 diagnostic pitfalls in this case that have not been previously described. Based on these data, a panel of markers and the use of sections containing nonneoplastic mesothelium to facilitate interpretation is recommended.

Low-Grade Endometrial Stromal Sarcoma and Undifferentiated Endometrial Sarcoma: A Comparative Analysis Emphasizing the Importance of Distinguishing Between These Two Groups

Bartosch, C., Exposito, M., Lopes, J. M. — Fri, 29 May 2009 01:54:44 PDT

Endometrial stromal sarcomas (ESSs) are rare tumors whose classification is still controversial. In this study, the authors studied 19 patients diagnosed with ESS at the Hospital S João, Porto, Portugal; reviewed their files and material; and performed immunohistochemical study for CD10, desmin, and smooth muscle actin markers, aiming to compare low-grade endometrial stromal sarcomas (LG-ESSs) and undifferentiated endometrial sarcomas (UESs) using the World Health Organization (WHO) classification. Twelve cases (63%) were classified as LG-ESS and 7 (37%) as UES. The median age at diagnosis was 49 years, and women with LG-ESS tended to be younger than those with UES. Most cases (7/11) had a previous echographic diagnosis of leiomyoma. A biopsy or curettage was performed in 6 cases, providing a definitive diagnosis of malignancy in 4. Frozen section was performed in 4 patients. The majority (63%) of patients were FIGO stage I. Twelve (63%) cases showed diffuse or focal expression of CD10. Desmin and smooth muscle actin expression was focal in 4 (21%) tumors. Patients with LG-ESS had a significant better overall survival than those with UES (P = .026). Mitotic count had no prognostic significance. Our data emphasize the clinical importance of the WHO classification in ESS. It is of utmost importance to establish a proper classification to increase the consistency of data that may be useful for improving clinical and therapeutic management of patients with ESS.

Skin-Type Adnexal Tumor With Trichoblastic Germinative Differentiationin the Breast: A Case Report

Mon, 25 May 2009 02:53:49 PDT

Adnexal tumors with follicular differentiation in the breast parenchyma are rarely encountered. The authors present a unique case arising in a 64-year-old woman, in whom they observed composite differentiation toward follicular germinative cells of the hair follicle with focal areas of outer root sheath differentiation and pilar-type keratinization. The histogenesis of this tumor is analyzed in light of the peculiar pathological, immunohistochemical, and molecular genetic findings.

Heterotopic Gastric Mucosa in the Cystic Duct

Orizio, P., Villanacci, V., Bassotti, G., Falchetti, D., Torri, F., Ekema, G. — Sat, 16 May 2009 06:57:22 PDT

Heterotopic gastric mucosa in the gallbladder is rare (with about 100 reported cases), and its occurrence is even rarer in the biliary ductal system. The authors report a pediatric case of heterotopic gastric mucosa in the cystic duct associated with cholelitiasis.

Symplastic Glomangioma: Information Review and Addition of a New Case

Arsenovic, N., Ramaiya, A., Moreira, R. — Fri, 15 May 2009 10:14:49 PDT

Glomus tumors are neoplasms differentiating toward specialized cells found in the glomus body, a structure involved in thermoregulation. Although the vast majority is benign, the occurrence of malignant glomus tumors is well recognized. Criteria for prediction of malignant potential include a deep location, large size, infiltration, necrosis, and nuclear atypia. Symplastic glomus tumors are benign neoplasms showing striking nuclear pleomorphism in the absence of other malignant criteria. Recognition of this rare morphologic aberration would prevent an inadvertent diagnosis of malignancy.

Primary Follicular Dendritic Cell Sarcoma of the Thyroid Gland Coexisting With Hashimoto's Thyroiditis

Yu, L., Yang, S. J. — Fri, 15 May 2009 10:14:49 PDT

Follicular dendritic cell (FDC) sarcoma, especially of extranodal origin, is an extremely rare malignancy of FDC origin, with only 1 case previously documented in the thyroid. The authors report the case of a 58-year-old female who presented with a painless mass in her neck. The neoplastic cells expressed monocyte/macrophagespecific marker CD68 (KP-1) and lysozymes and the dendritic cell-associated antigens CD35 and Fascin but was negative for CD1a, CD21, and CD23, most consistent with a diagnosis of FDC sarcoma. BIOMED-2 multiplex polymerase chain reaction analysis showed B-cell clonality in both tumor and its adjacent coexisting Hashimoto’s thyroiditis. To the authors’ knowledge, this is the first report of a rare entity of FDC sarcoma primarily involving the thyroid gland coexisting with Hashimoto’s thyroiditis.

Predicting the Likelihood of Additional Nodal Metastases in Breast Carcinoma Patients With Positive Sentinel Node Biopsy

MustaC, E., Matuan-Ilija, K., MarijiC, B., Smokvina, M., JonjiC, N. — Fri, 15 May 2009 10:57:24 PDT

Axillary lymph node dissection (ALND) is an important procedure in the staging of breast cancer patients. However, it is associated with a significant morbidity rate. In addition, using early diagnosis a high number of cases with negative lymph nodes can be identified. A lymph node defined as sentinel lymph node (SLN) would be the first to receive tumoral drainage. A less morbid but accurate staining procedure using mapping and SLN biopsy has been introduced. The aim of this study was to estimate the likelihood of additional disease in the axilla after SLN analysis. A total of 259 breast carcinomas and SLN biopsies followed by ALND were examined. The patient median age was 59 years, approximately 75% of them postmenopausal. Tumor size was 1.4 ± 0.8 cm (almost 80% in pT1). SLNs were positive in 59 of 259 (22.8%) carcinomas, 30 (11.6%) with micrometastases (<2.0 mm) and 29 (11.2%) with metastases. Tumor size (P = .004) and presence of lymphovascular invasion (LVI; P = .034) were found to be significant predictors of pathologically positive SLN. Following ALND, positive non-SLNs were present mostly in patients with metastasis >2 mm in SLN (P = .003), in carcinoma with higher nuclear grade (P = .044), decreased estrogen receptor (ER; P = .042), and progesterone receptor (PR; P = .042). Finally, lymph node status (pN) following SLN and ALND was found to be significantly associated with tumor size (P = .006), LVI (P = .037), PR (P = .023), and Her-2 status (P < .001). These results point to detailed analysis of primary tumor and SLN that may increase the precision of patient selection for further axillary surgery or radiotherapy.

Colonic Carcinoma Metastatic to the Endometrium: The Importance of Clinical History in Averting Misdiagnosis as a Primary Endometrial Carcinoma

Zannoni, G. F., Vellone, V. G., Fadda, G., Petrillo, M., Scambia, G. — Thu, 14 May 2009 16:11:39 PDT

Metastases to endometrium are a rare event. A case of a 53-year-old woman with a previous colon cancer presenting with a dysfunctional uterine bleeding is reported. The curettage specimens consisted of multiple fragments of proliferative endometrial tissue mixed with irregular glands, focally amputated, lined by very cells with elongated, hyperchromatic nuclei and solid sheets of neoplastic cells often filled with necrotic debris and inflammatory cells, resembling endometrioid adenocarcinoma. Neoplastic cells resulted positive at immunohistochemical reaction for CK20, CDX2, p53 and negative for CK7, vimentin, ER, and PR suggesting a metastatic colon adenocarcinoma. Absence of premalignant changes, such as hyperplasia, besides frankly malignant glands in an endometrial curette should be regarded as an alarming feature for a secondary nature mostly from large bowel. In these cases clinical history is crucial and an immunohistochemical panel made up of CK7, CK20, CDX2, p53, vimentin, ER, and PR is useful for the correct diagnosis.

Pgp 9.5 and Cyclin D1 Co-Expression in Cutaneous Squamous Cell Carcinomas

Mastoraki, A., Ioannidis, E., Apostolaki, A., Patsouris, E., Aroni, K. — Thu, 14 May 2009 16:11:40 PDT

Background: squamous cell carcinoma (SCC) consists of altered keratinocytes, presents variable differentiation, inexorably progresses, and on occasion metastasizes. Objective: to investigate the biological activity of epidermal cells in SCCs by estimating the expression of PGP 9.5 and cyclin D1 using immunohistochemistry. Methods: the sample included 13 well-differentiated cases of cutaneous SCC (grade I), 12 moderately differentiated tumors (grade II), and 7 poorly differentiated lesions (grade III). Four cases belonged to the distinct entity of pseudoadenoid SCC. Results: PGP 9.5 expression was positively correlated with tumor stage (P < .001) and potential perineural invasion (P < .001), whereas cyclin D1 expression correlated inversely with the degree of cellular differentiation (P < .001) and PGP 9.5 immunostaining (P < .001). Conclusion: PGP 9.5 and cyclin D1 coexpression was closely associated with tumor aggressiveness and can be classified as a marker for predicting the outcome of resection-treated skin cancer patients.

Lynch Syndrome-Related Endometrial Carcinomas Show a High Frequency of Nonendometrioid Types and of High FIGO Grade Endometrioid Types

Carcangiu, M.L., Radice, P., Casalini, P., Bertario, L., Merola, M., Sala, P. — Thu, 14 May 2009 16:11:39 PDT

Although endometrial cancer (EC) represents the second most common cancer after colonic cancer in patients with Lynch syndrome (LS), the pathologic features and behavior of LS-related EC are not well recognized. ECs from 23 patients (mean age 46.2 years) with MSH2 (16), MLH1 (6), and MLH1/MSH2 (1) constitutional mutations were evaluated for histologic type, FIGO grade, FIGO stage, association with tumors in other sites, and survival. For every LS-associated tumor, 2 same-age cases from patients with no family history of LS-associated cancer were evaluated (sporadic group). In LS-associated EC there were 13 (56.5%) endometrioid (eec) and 10 (43.5%) nonendometrioid carcinomas (neec), and among the sporadic tumors there were 44 (95.7%) eec and 2 (4.3%) neec (P = .001). The eec in women with germline LS mutation had a significantly higher FIGO grade (P = .0378) and more frequent vascular invasion than the controls. The mean survival of the entire group of 23 patients with LS-related EC was 17.326 (14.066 to 20.585). Mean survival according to FIGO stage was significant (P = .010). Difference in mean survival according to age of the patient at the time of the diagnosis (patients >46 years vs <46 years) was not significant. The mean survival of the mutated patients with eec was 20.462 (17.564 to 23.359) and was not significantly different from that of the mutated patients with neec, which was 14.240 (9.119 to 19.361). Log-rank analysis showed that histology did not affect the survival. However, the hazard ratio of neec patients, although not significant, resulted higher. Mean survival of patients with a neec tumor combined with an endometrioid component (14.375 [8.084 to 20.666]) was not different from that of patients with pure neec cancers (14.250 [7.885 to 20.615]). When compared with the control population, LS-related ECs show a wider variety of histologic types; a higher frequency of neec types despite the younger age of the patients, and a higher frequency of high grades among the eec.

Chromosome 7 Aneusomy in Metaplastic Breast Carcinomas With Chondroid, Squamous, and Spindle-Cell Differentiation

Gwin, K., Lezon-Geyda, K., Harris, L., Tavassoli, F. A. — Fri, 01 May 2009 02:04:26 PDT

Metaplastic breast carcinomas (MBCs) are basal-like tumors that frequently express epidermal growth factor receptor (EGFR) via an unknown underlying genetic mechanism. In this study, the EGFR/CEP7 gene copy number in 17 MBCs with chondroid, squamous, and spindle-cell differentiation showing EGFR expression by immunohistochemistry was analyzed using fluorescence in situ hybridization. All cases had a balanced EGFR/CEP7 ratio. EGFR gene amplification was not observed in any case. Monosomy was found in 25% and polysomy in 12.5% of carcinomas with chondroid differentiation. All spindle-cell carcinomas and 50% of squamous carcinomas showed trisomy. Comparison with CEP7 copy number revealed aneusomy of chromosome 7, as opposed to increases or decreases specific to the EGFR gene or 7p. Although no direct correlation between EGFR expression by immunohistochemistry and aneusomy was observed, cases with a score of 3+ showed a higher frequency of EGFR gene copy gain. In the absence of EGFR amplification, chromosome 7 aneusomy might be a useful criterion for the determination of potential candidates for EGFR tyrosine kinase inhibitor clinical trials.

Metastatic Clear Cell Renal Cell Carcinoma Presenting as Multiple Colonic Polyps

Chetty, R., Syed, A., Kwast, T. v. d. — Fri, 01 May 2009 02:04:26 PDT

A 92-year-old male presented for routine endoscopic surveillance of his gastrointestinal (GI) tract. He did not have any GI symptoms currently, and the patient had undergone a right nephrectomy for renal cell carcinoma 17 years previously. A lower GI endoscopy revealed polyps in the ascending colon, hepatic flexure, and sigmoid colon (2 polyps). All the polyps were snared and removed in toto. Histological evaluation of all 4 polyps showed similar features. There was expansion of the lamina propria by sheets of clear cells arranged in a nested pattern with a rich vascular network. Immunohistochemistry showed the tumor to be positive for low-molecular-weight cytokeratin, CD10, and vimentin. The features were morphologically and immunophenotypically that of clear cell renal cell carcinoma. This case highlights an extremely unusual presentation of recurrent renal cell carcinoma as multiple, separate colonic polyps 17 years after resection of the primary tumor.

Autopsy Findings in 14 Patients With Penile Squamous Cell Carcinoma

Chaux, A., Reuter, V., Lezcano, C., Velazquez, E., Codas, R., Cubilla, A. L. — Fri, 01 May 2009 02:04:25 PDT

The aim of this study was to describe pathologic features found at autopsy of 14 patients with penile cancer. Nine patients died from disseminated disease; 5 of them presented local/regional recurrences. Five patients died from other causes, 2 of them postoperatively. Local recurrence sites were corpus cavernosum, Buck’s fascia and urethra, regional skin, and prostate. Metastatic sites were lymph nodes (9 cases), liver (7 cases), lungs (6 cases), heart (5 cases), adrenals, bone and skin (3 cases each), thyroid and brain (2 cases each), and pancreas, spleen, and pleura (1 case each). Patients with heart metastasis had arrhythmias. Patients who died and who did not die from penile cancer had different profiles: low-grade superficial tumors with usual and warty subtypes versus high-grade deeply invasive basaloid or hybrid verrucous/sarcomatoid carcinomas. A natural history model for penile cancer routes of spread is proposed: local intrapeneal, regional and systemic nodes, regional skin, liver, lungs, heart, and other multiple sites.

Dedifferentiated Epithelial-Myoepithelial Carcinoma of the Parotid Gland With Aberrant Expression of Prostate Specific Antigen: A Case Report

Sarode, V. R., Truelson, J., Zaidie, M. — Wed, 29 Apr 2009 01:10:56 PDT

This report describes a rare case of dedifferentiated epithelial–myoepithelial carcinoma (EMCa) of the parotid gland. The tumor had 2 distinct components; a well-differentiated EMCa comprising of ductal and myoepithelial cells with low nuclear grade and low proliferation index. The second component showed a completely different morphology comprising of sheets and clusters of poorly differentiated cells with high nuclear grade and proliferative activity. The dedifferentiation was associated with an accelerated clinical course. Dedifferentiated EMCa is extremely rare with only 8 cases reported in the English literature. An unusual feature, hitherto not described in this tumor is the aberrant expression of prostate-specific antigen in the dedifferentiated component. In male patients, this may cause diagnostic confusion with metastatic prostate carcinoma.

Absence of Peritumoral Fibrosis or Inflammatory Infiltrate May Be Related to Clinical Progression of Papillary Thyroid Microcarcinoma

Wed, 29 Apr 2009 01:10:55 PDT

Rio Grande do Norte (RN) shows the highest relative incidence of papillary carcinomas in Brazil. To analyze histological features that might be associated with this incidence, the authors compared thyroid glands from 463 autopsies performed in RN with 427 surgical and autopsy glands previously studied in Sao Paulo (SP). The authors found 41 papillary thyroid microcarcinomas (PTMs) in 35 glands (8.1%), an incidence similar to the one reported in SP (7.8%). However, PTMs were predominantly nonencapsulated nonsclerosing at microscopy (44.0%), in contrast with SP where these types of lesion represented only 4 out of 32 PTMs (12.5%; P = .0046). The authors suggest that these nonencapsulated lesions with no sign of inflammation may represent an early stage that may evolve to clinical cancers, contributing to the high incidence of clinically differentiated thyroid carcinomas observed in RN.

Angiogenesis in the Progression of Breast Ductal Proliferations

Carpenter, P. M., Chen, W.-P., Mendez, A., McLaren, C. E., Su, M.-Y. — Wed, 29 Apr 2009 01:10:55 PDT

Angiogenesis, the formation of blood vessels, is necessary for a tumor to grow, but when angiogenesis first appears in the progression of breast ductal carcinomas is unknown. To determine when this occurs, the authors examined microvessel density (MVD) by CD31 and CD105 immunostaining in normal ducts, 32 cases of usual hyperplasia, 19 cases of atypical hyperplasia, and 29 cases of ductal carcinoma in situ (DCIS). Simple hyperplasia had a 22-fold greater MVD than normal ducts (P < .0001). An increase during the progression of ductal changes was highly significant (P < .0001). To determine a possible mechanism, immunohistochemistry for vascular endothelial growth factor (VEGF) was evaluated. VEGF staining intensity of ductal epithelium increased during the progression from normal to hyperplastic to DCIS. This study shows that the first significant increase in angiogenesis occurs very early in the evolution of ductal proliferations as ductal cells become hyperplastic.

Distinctive Morphology of Renal Cell Carcinomas in Tuberous Sclerosis

Wed, 29 Apr 2009 01:10:55 PDT

Tuberous sclerosis complex results from mutations in 1 of 2 interacting gene products, hamartin or tuberin. The syndrome is characterized by hamartomas and neoplastic lesions, including angiomyolipomas of the kidney and other organs. Renal cell carcinoma (RCC) in tuberous sclerosis remains relatively poorly characterized because historical studies were confounded by the inclusion of epithelioid angiomyolipomas. The authors present a patient with tuberous sclerosis and bilateral renal lesions, including multiple minute angiomyolipomas, cortical cysts, and 4 separate RCCs of unclassified type. The carcinomas shared distinctive morphological features, including sheet-like, glandular, trabecular, or cystic architecture and abundant granular eosinophilic cytoplasm. By definition, the carcinomas were keratin positive and negative for HMB-45 and Melan-A. Detailed immunohistochemical analysis revealed heterogeneity among the cortical cysts and carcinomas. The histopathological features of these carcinomas illustrate characteristics of renal carcinoma that are probably related to genetic alterations of tuberous sclerosis.

A Case of Juxtaglomerular Cell Tumor With Novel Histologic Features

Onder, S., Ertoy Baydar, D. — Wed, 29 Apr 2009 01:10:56 PDT

Juxtaglomerular cell tumors constitute a very small group among the renal neoplasms. Most secrete renin, causing hypertension and hypokalemia due to secondary hyperaldosteronism. They have characteristic pathological features. For the accurate evaluation, diagnosis has to be confirmed by immunohistochemical staining for renin or electron microscopic demonstration of renin protogranules. This report presents a case of renal juxtaglomerular cell tumor in a 63-year-old man with previously unreported histological findings in addition to classic morphology.

Composite Biclonal Marginal Zone Lymphoma of Lung and Chronic Lymphocytic Leukemia in a Patient: Pathologic, Phenotypic, Cytogenetic, and Molecular Study

Wed, 15 Apr 2009 21:09:46 PDT

The simultaneous diagnosis of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is rare. This study reports a patient with composite synchronous biclonal occurrence of MALT lymphoma of the lung and CLL/SLL. The morphology of the lung and peripheral blood showed features of MALT lymphoma and CLL, respectively. The cytogenetic evaluation of the lung specimen revealed a t(1;14)(p22;q32), a frequent genetic abnormality in MALT lymphoma. Flow cytometry analysis of the lung tissue showed features of MALT lymphoma and CLL/ SLL with different light chain restriction, whereas the blood showed phenotypic evidence of CLL/SLL. Fluorescence in situ hybridization study of the blood showed a deletion of 13q14 and 17p13. Immunoglobulin heavy chain (IgH) gene rearrangement study of the lung tissue and blood showed a monoclonal IgH gene rearrangement with distinct light chain restriction, suggesting that the immunophenotypically different cell populations originated from separate clones.

Basal-Like Subtype Breast Cancers in Women Older Than 40 Years of Age

Ersahin, C., Chivukula, M., Bhargava, R., Dabbs, D. — Wed, 15 Apr 2009 21:09:47 PDT

Basal-like (BL) carcinoma, distinguished by the expression of keratins that are a characteristic of myoepithelial cells, is 1 of the 5 distinct subtypes of breast tumors identified by gene microarray technologies. BL cancers have been well described in women <40 years of age. However, little data exist about this carcinoma in older patients. Twenty-three BL breast cancer specimens from patients >40 years of age were evaluated. The study demonstrated that there is a subset of patients >40 years of age with breast cancers, manifesting features of BL carcinoma. There has been a significant increase in BL cancers among women >40 years of age having larger tumors and lymph node metastasis in comparison with younger women <40 years of age. This could be due to the tumor heterogeneity in BL cancers between the 2 age groups. BL cancer patients from all age groups require further investigation for BRCA-1, as well as gene microarray analysis to compare the gene expressions with those observed in the younger population.

The Clinical Significance of Neuroendocrine Differentiation in T3-T4 Node-Negative Colorectal Cancer

Wed, 15 Apr 2009 21:09:45 PDT

This study was conducted to determine the clinical significance of neuroendocrine differentiation in cases of T3–T4 node-negative colorectal cancer. Eighty-nine patients diagnosed with T3–T4 node-negative colorectal cancer who underwent curative resection were enrolled. Tumors expressing neuroendocrine markers were classified as either low expression (≤2% cells staining positive for a neuroendocrine marker) or high expression (≤2% cells staining positive for a neuroendocrine marker). Immunohistochemical staining for chromogranin A and synaptophysin revealed high expression in 27 (30.3%) and 69 (77.5%) of the 89 patients, respectively. All tumors that showed high expression of chromogranin A also displayed high expression of synaptophysin. With the exception of preoperative carcinoembryonic antigen, no statistically significant correlation was found between neuroendocrine differentiation and all other clinicopathologic variables. Analysis using the Kaplan–Meier method and multivariate Cox regression model demonstrated that neuroendocrine differentiation for chromogranin A and synaptophysin was not associated with disease-free survival. Therefore, neuroendocrine differentiation markers would not be useful variables for prognostic assessment of patients with T3–T4 nodenegative colorectal cancer.

IgG4-Related Disorder of the Retroperitoneum Resembling Castleman's Disease Plasma Cell Type: A Report of 2 Cases

Kojima, M., Nakamura, N., Motoori, T., Shimizu, K., Haratake, J., Nakamura, S. — Wed, 15 Apr 2009 21:09:46 PDT

This study reports 2 cases of IgG4-related disorder of the retroperitoneum resembling plasma cell type of Castleman’s disease. A single lesion was located in the renal hilum and ureter, respectively, in these 2 cases. Histologically, both lesions were characterized by reactive follicular hyperplasia with active germinal centers and a sheet of polyclonal mature plasma cells in the interfollicular area. The prominent sclerosis and/or fibrosis, which were characteristic histological findings of IgG4-related disorders, were absent. However, immunohistochemical study demonstrated numerous IgG4+ plasma cells accounting for more than 50% of IgG+ cells. The phlebitis that occurs with early lesions of obliterative phlebitis is one of the characteristic histological findings of IgG4-related disorders and was noted in one case. Serum IgG4 concentration was increased in one case. The serum interleukin-6 level was within the normal range in one case that was examined. From a therapeutic perspective, it is important to discriminate IgG4-related disorder from plasma cell type of Castleman’s disease.

Breast Carcinoma With Chondroid Differentiation: A Clinicopathologic Study of 21 Triple Negative (ER-, PR-, Her2/neu-) Cases

Gwin, K., Wheeler, D. T., Bossuyt, V., Tavassoli, F. A. — Wed, 11 Mar 2009 21:07:42 PDT

A total of 21 metaplastic breast carcinomas (MCs) with chondroid differentiation were evaluated to better establish the clinicopathological features of this variant. The tumors exhibited mainly invasive carcinoma admixed with areas of cartilaginous matrix production. An associated ductal intraepithelial neoplasia component grade 2 or 3 was observed in 43% of cases. Immunohistochemical analysis revealed a triple negative (ER-, PR-, and Her2/ neu-) immunoprofile and no expression of the androgen receptor. EGFR-positivity was found in 88% of evaluated cases, consistent with the proposed basaloid phenotype for all MC. Compared with previous studies that reviewed MC with osseus and cartilaginous elements, the incidence of axillary lymph node metastasis was significantly higher in our study and 60% of positive nodes exhibited chondroid differentiation. Available follow-up data (n = 10) revealed aggressive behavior of this MC variant with frequent metastasis, including visceral involvement and local chest wall recurrence despite chemotherapy and radiation. Three patients subsequently died of metastatic disease.

Endosalpingiosis of the Urinary Bladder: A Case of Probable Implantative Origin With Characterization of Benign Fallopian Tube Immunohistochemistry

Maniar, K. P., Kalir, T. L., Palese, M. A., Unger, P. D. — Wed, 11 Mar 2009 21:07:42 PDT

Müllerianosis of the bladder is an infrequently described lesion consisting of multiple Müllerian-type tissues within the urinary bladder. Few previous cases of pure endosalpingiosis have been described. Here we present a 54-yearold post-menopausal female with a history of prior pelvic surgery with traumatic bladder injury, who was found to have a cystic lesion in the posterior wall of the bladder. Routine histology demonstrated cyst epithelium characteristic of endosalpingiosis. Three benign Fallopian tube specimens were obtained and stained with the relevant immunohistochemical markers for comparison. Results showed an identical immunohistochemical profile between the bladder cyst lining and the normal Fallopian tube controls. This case represents a rare instance of pure endosalpingiosis of the urinary bladder, with a likely implantative origin. This form of bladder Müllerianosis should therefore be considered within the differential diagnosis of cystic lesions of the bladder.

Mature Solid Teratoma of the Fallopian Tube Mimicking Metastasis of Endometrial Adenocarcinoma: A Case Report

Roncati, L., Barbolini, G., Ghirardini, G., Rivasi, F. — Wed, 11 Mar 2009 21:07:42 PDT

We report a mature solid teratoma of the fallopian tube in an elderly woman. This mass was noted on CT scan and considered metastatic in nature since following a bioptical diagnosis of endometrial adenocarcinoma. Hysterectomy and bilateral salpingectomy and ovariectomy were performed and a second minor mature solid teratoma was discovered inside the right ovary. Neoplasms of the fallopian tube are very uncommon and this is the first Italian case to be added to about other 60 of the literature. Moreover it joins minimum teratomatous diameter with maximum recorded age.

Primary Pulmonary Osteosarcoma: A Report of Four Cases and a Review of the Literature

Shenjere, P., Travis, W. D., Franks, T. J., Doran, H. M., Hasleton, P. S. — Wed, 11 Mar 2009 21:07:41 PDT

Primary pulmonary osteosarcoma is very rare. Most cases are secondary deposits from primaries arising in the appendicular skeleton. Four cases of primary osteogenic sarcoma of the lung are described and the literature reviewed for previously reported cases. These pulmonary tumors occur in patients who are in their fourth to seventh decades, that is, an older age group than their primary bone equivalent. There is a slight male predominance. There appears to be a propensity for the left lung, especially the left upper lobe. The clinical presentation is similar to primary (epithelial) lung cancer. Differentiation from pleomorphic carcinomas and other sarcomas is discussed. We know of no predisposing factor(s) in our cases for the development of this tumor.

Hepatic Angiomyolipoma and Intramural Small Intestinal Schwannoma: A Coincidence or a Relationship?

Marino Marsilia, G., Boscaino, A., Mura, A. L., Ceriello, A., Ritis, R. D. — Wed, 11 Mar 2009 21:07:41 PDT

We report a case of hepatic angiomyolipoma associated to a small bowel schwannoma in a 40-years old woman. Both lesions were asyntomatic. Histologically, hepatic angiomyolipoma showed oncocytic features and scanty adipose tissue, the tumor cells expressed desmin, smooth muscle actin, S-100 protein and HMB45. The tumor cells of intramural small intestinal mass were positive for S-100 protein and GFAP and negative for CD117, CD34 and desmin. To the best of our knowledge, no case of hepatic angiomyolipoma has been previously reported in association with intestinal schwannoma.

Thymolipoma, Report of a Case Suggesting an Origin From Thymic True Hyperplasia

Kondo, T., Masuya, D., Mukai, K. — Thu, 19 Feb 2009 23:21:37 PST

The authors describe a rare case of thymolipoma in a 26-year-old, otherwise healthy woman presenting with general fatigue. Radiological examinations disclosed a large anterior mediastinal mass. Macroscopically, the resected tumor was approximately 5 x 10 cm², with nodular configuration, and microscopically, 3 components were identified: a normal thymic rim, hyperplastic thymic tissue, and a typical thymolipomatous component at the periphery of the hyperplastic area. The predominant component of the tumor was hyperplastic thymic tissue. The tumor was diagnosed as thymolipoma at the initial stage of development. This case suggests the histogenesis of thymolipoma—fatty regression or infiltration of fatty tissue into a previously hyperplastic thymus.

Adenoid Cystic Carcinoma of the Breast Associated With Invasive Duct Carcinoma: A Case Report

Thu, 19 Feb 2009 23:21:37 PST

Interdigitating Dendritic Cell Tumor of the Lymph Node in the Right Submaxillary Region: A Case Report and Review of the Literature

Zhou, Z.-y., Sun, R.-c., Yang, S.-d., Pan, M.-h. — Tue, 17 Feb 2009 05:34:43 PST

Interdigitating dendritic cell tumor/sarcoma is an extremely rare neoplasm that mainly occurs in the lymph node, with only 51 cases reported in the literature to date. The authors report the case of a 41-year-old woman who presented with a 4-month history of a gradually enlarging painless mobile lymphadenopathy in the right submaxillary region. The lymph node mass was completely resected and was treated with 1 cycle of CHOP chemotherapy. After 10 months, she was alive with no evidence of disease. Because interdigitating dendritic cell sarcomas are rare and can show morphologic and immunohistochemical heterogeneity, correct diagnosis requires a high index of suspicion and complete pathological study.

Intraoperative Consultation for Axillary Sentinel Lymph Node Biopsy: An 8-Year Audit

Tue, 17 Feb 2009 05:34:42 PST

To summarize the authors’ 8-year institutional experience with intraoperative consultation via frozen section (FS) on sentinel lymph node biopsy (SLNB) in breast cancer patients we recorded the, complete operative procedure including additional surgery on the ipsilateral axilla and intraoperative consultation and permanent histopathologic processing for all cases with inoperative consultation on SLNB in breast cancer patients between the groups, ² and Fisher’s exact tests were used. Intraoperative consultation was positive in 116/706 cases (16.4%) and final pathology in 158/706 cases (22.4%); the false-negative rate was 26.6%, the false-positive rate was 0%, and the overall accuracy was 94%. False-negative rate was significantly associated with the size of metastasis (micro vs macrometastasis; P < .002) but not significantly associated with the histologic type (P = 0.76) or pathologist expertise (P = 0.08). The rate of spared second procedures was 92% when calculated exclusively for patients who ultimately underwent ALND. Intraoperative consultation via FS for SLNB is a safe practice that can reliably save clinically node-negative patients a second surgery.

Fuzeon-Induced Collagenophagic Granuloma: A Peculiar Granulomatous Injection Site Reaction to Fuzeon--A Case Report and Review of Literature

Sidhu, H. K., Tvetenstrand, C. D., Chaffee, B. H., Sidhu, J. S. — Tue, 17 Feb 2009 05:34:42 PST

Enfuvirtide (ENF, T-20, or Fuzeon [Hoffman-La Roche Inc, Nutley, NJ, and Trimeris, Inc, Durham, NC]) is an HIV-1 fusion inhibitor and is the only injectable antiretroviral drug available. Injection site reactions (ISRs) are the most frequently reported adverse events, occurring in about 98% of patients. A granuloma annulare–like granulomatous ISR has been reported. We report a granulomatous ISR that is different from granuloma annulare and granuloma annulare–like reaction because it is rich in multinucleated giant cells engulfing altered collagen. We call this type of ISR a collagenophagic granuloma. Most previous reports—with the exception of 1 report—about ISRs with ENF treatment have used punch biopsies, which lack the depth to analyze the reticular dermis and subcutaneous tissue and, therefore, may have missed ISRs, which look like granuloma annulare, and the collagenophagic granulomatous reaction.

The Value of CD23 Expression as an Additional Marker in Distinguishing Mediastinal (Thymic) Large B-Cell Lymphoma From Hodgkin Lymphoma

Salama, M. E., Mariappan, M. R., Inamdar, K., Tripp, S. R., Perkins, S. L. — Tue, 17 Feb 2009 05:34:44 PST

Mediastinal diffuse large B-cell lymphoma (Med-DLBCL) is a subtype of DLBCL that has morphologic and clinical similarities and phenotypic overlaps with classical Hodgkin lymphoma (CHL) involving the mediastinum. CD23 is a marker that has been previously reported in Med-DLBCI and is proposed in the differential diagnosis of M-DLBCL and CHL. A panel of immunostains, including CD45, CD20, CD3, CD30, CD15, CD21, and CD23 as well as Eber was performed on Med-DLBCL and 20CHL. 23/27 Med-DLBCL (85%) were positive for CD23 (membranous) CD30 was negative in 6 and positive in 21 cases. 18 CHL cases were negative for CD23 and only 2 showed rare scattered Reed–Sternberg cells with weak cytoplasmic CD23 staining. CD23 showed a sensitivity of 85% and positive predictive value of 92%. In conclusion CD23 is a useful marker in distinguishing Med-DLBCL and CHL in mediastinal biopsies and may be helpful as an adjunct to histomorphology and other markers in the diagnosis and appropriate clinical management of these lesions.

Inflammation of Ectopic Pancreatic Tissue and Presence of Other Ectopic Tissues in a Meckel's Diverticulum Causing Acute Abdominal Symptoms: A Case Report and Review of the Literature

Zarand, A., Bajtai, A., Baranyai, Z., Dede, K., Jakab, F. — Tue, 17 Feb 2009 05:34:43 PST

An unusual case of acute abdomen was caused by the inflammation of ectopic pancreatic tissue in a Meckel’s diverticulum. A 49-year-old man presented with acute abdominal pain, and the clinical diagnosis of acute appendicitis was established. During laparotomy, a normal appendix of unusual localization near the gallbladder and a Meckel’s diverticulum with an inflamed tip were found. Histological examination showed acute inflammation of heterotopic pancreatic tissue along with normal ectopic gastric and duodenal mucosa within the wall of the diverticulum. Fat necrosis was also ascertained. The authors believe that this is the first report of acute inflammation of ectopic pancreatic tissue and the presence of normal ectopic gastric and duodenal tissue in the same Meckel’s diverticulum.

Mastocytosis of the Female Breast

Setia, N., Crisi, G. M., Pantanowitz, L. — Tue, 17 Feb 2009 05:34:42 PST

A subset of patients with systemic mastocytosis may manifest with extracutaneous involvement. To the best of our knowledge, mastocytosis of the human breast has not been described. This study reports a case with mastocytosis involving the breasts of a 33-year-old woman associated with mammary hypertrophy (breast mastocytosis). The potential for infiltrating mast cells to mimic lobular carcinoma is emphasized and the relationship to breast hypertrophy in this case is discussed.

The Expression and Clinical Significance of TGF-B1 and MMP2 in Human Renal Clear Cell Carcinoma

Yang, S. d., Sun, R.-C., Mu, H.-j., Xu, Z.-q., Zhou, Z.-y. — Tue, 17 Feb 2009 05:34:42 PST

The expression and clinical significance of transforming growth factor B1 (TGF-B1) and matrix metalloproteinase-2 (MMP2) in human renal clear cell carcinoma (RCCC) were investigated. The intensity of TGF-β and MMP2 expression in RCCC kidneys was significantly higher than that in normal kidneys. Expression of TGF-B1;1 and MMP2 in RCCC tissues was positively correlated with pathological grade and clinical stage. There was also a significant correlation between TGF-B1 and Msshese analyses indicate that upregulation of TGF-B1 and MMP2 expression may occur during the progression of RCCC. Thus, TGF-B1 and MMP2 may be useful molecular markers for evaluating prognosis in RCCC patients.

Warthin Tumor of the Upper Lip: An Unusual Location

Almeida, A. d. S., Hanemann, J. A. C., Oliveira, D. T. — Tue, 03 Feb 2009 02:12:04 PST

Warthin tumor (papillary cystadenoma lymphomatosum) is a benign salivary gland tumor involving almost exclusively the parotid gland. The lip is a very unusual location for this type of tumor, which develops only rarely in minor salivary glands. The case of 42-year-old woman with Warthin tumor arising in minor salivary glands of the upper lip is reported.

Glomus Tumor of the Kidney: Case Report and Literature Review

Sasaki, K., Bastacky, S. I, Hrebinko, R. L, Parwani, A. V, Zynger, D. L — Wed, 28 Jan 2009 02:44:25 PST

Glomus tumor is a benign mesenchymal neoplasm of the subcutaneous tissue of the distal extremities and head and neck region. Glomus tumor rarely occurs in the visceral organs. This study reports the sixth case of a glomus tumor arising in the kidney in a 62-year-old man who presented with weight loss and an incidental kidney lesion detected by computed tomographic scan. Radiologically, the tumor was difficult to differentiate from a malignant lesion and was therefore excised by partial nephrectomy. The tumor was challenging to diagnose by routine hematoxylin and eosin microscopic examination, necessitating immunohistochemical analysis. Immunoreactivity was demonstrated for smooth muscle actin, vimentin, collagen IV, and CD57, with little to no expression of neuroendocrine, endothelial, or epithelial markers. To date, the tumor has followed a benign course without evidence of local recurrence or metastasis.

Thyroid Involvement in Disseminated Zygomycosis by Cunninghamella bertholletiae: 2 Cases and Literature Review

Mayayo, E., Klock, C., Goldani, L. — Sat, 24 Jan 2009 22:37:53 PST

Abstract: Thyroid involvement is very rare in disseminated zygomycosis. We describe two cases of thyroiditis due to Cunninghamella bertholletiae diagnosed though a necropsy study and the only five previous cases of thyroid zygomycosis reported in the literature are revised.

Xp11.2 Translocation Renal Carcinoma With Placental Metastasis: A Case Report

Bovio, I. M., Allan, R., Oliai, B., Hampton, T., Rush, D. — Thu, 22 Jan 2009 03:56:50 PST

Renal cell carcinomas with sporadic Xp11.2 translocations are uncommon malignancies in children and young adults associated with several different reciprocal translocations involving the TFE3 gene located on chromosome Xp11.2. Placental metastases are extremely rare, with only a handful of cases reported. This study reports the case of a 20-year-old woman with an Xp11.2 translocation renal carcinoma that metastasized to the placenta. This is the first reported case of a renal cell carcinoma metastatic to the placenta and highlights the aggressive behavior of Xp11 translocation renal cell carcinomas.

Fatal Cokeromyces recurvatus Pneumonia: Report of a Case Highlighting the Potential for Histopathologic Misdiagnosis as Coccidoides

Ryan, L. J., Ferrieri, P., Powell, R., Zeki, S., Pambuccian, S. — Wed, 14 Jan 2009 22:31:23 PST

Cokeromyces recurvatusis a dimorphic zygomycete with histologic morphology similar to Coccidioides immitis. A 66-year-old man who was status-post bone marrow transplantation for chronic myelogenous leukemia was hospitalized with new onset rash, nausea, and vomiting and subsequently expired. A sputum culture collected on the day of death revealed heavy growth of C. recurvatus 6 days after collection. At autopsy, microscopic examination of the lungs revealed numerous thick-walled, nonbudding spherules ranging in size from 40 to 80 µm. Initial immunohistochemical staining of the formalin-fixed lung tissue was positive for Coccidioides. Additional immunoperoxidase staining revealed the organisms were consistent with a zygomycete fungus, compatible with C. recurvatus infection. Polymerase chain reaction using panfungal primers was attempted on the formalin-fixed tissue but was inconclusive. This case highlights the potential for misdiagnosing Cokeromyces as Coccidioides when the diagnosis is based on histology and immunohistochemical staining.

Pulmonary Sarcomatoid Carcinomas: A Practical Overview

Wed, 14 Jan 2009 22:31:24 PST

Pulmonary sarcomatoid carcinomas (PSCs) are currently defined as poorly differentiated non-small-cell carcinomas containing a component with sarcoma or sarcoma-like (spindle and/or giant cell) features. They consist of 5 major histological variants, namely pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The segregation of PSCs into a distinct clinicopathologic entity seems justified on the basis of morphologic, behavioral, and genotypic/phenotypic attributes. As a group, PSCs generally run an aggressive clinical course and may cause major difficulties in the differential diagnosis with other primary and secondary malignancies of the lung. At present, PSCs are believed to represent a family of carcinomas "in transition," in which diverse pathways of clonal evolution account for histological differences of a common ancestor lesion. The sarcomatous or sarcomatoid component of these tumors is thought to derive from carcinoma cells during the progression of carcinogenesis through the activation of an epithelial–mesenchymal transition program leading to sarcomatous transformation or metaplasia (conversion paradigm). Conceivably, targeting the epithelial–mesenchymal transition program could become a valid therapeutic strategy for these life-threatening tumors, whose sensitivity to current medical manipulation is disappointing.

Bona Fide Primary Merkel Cell Carcinoma of an Intraparotid Lymph Node in a HIV-Positive Patient

Wed, 14 Jan 2009 22:31:22 PST

Merkel cell carcinomas are uncommon malignant tumors thought to originate from the neuroendocrine cells of the skin that mainly affects sun-exposed body areas, particularly the head and neck. In approximately 10% of cases, they present with localized lymphadenopathy without any clinical evidence or history of a primary lesion, but a truly primary lymph node Merkel cell carcinoma may occur, possibly originating from epithelial inclusions or an anomalous neuroendocrine differentiation of hematopoietic stem cells. It has been observed that Merkel cell carcinoma is more likely to affect patients whose immune status is impaired as a result of iatrogenic immunosuppression, human immunodeficiency virus infection, or hematological malignancies. This study reports the case of a bona fide primary Merkel cell carcinoma arising in an intraparotid lymph node of a patient infected by HIV that had a particularly unfavorable clinical course.

Squamous Metaplasia on the Breast Implant Capsule

Alikhan, M. B., Nassar, A., Mansoor, I. — Wed, 14 Jan 2009 22:31:23 PST

The development of squamous metaplasia or carcinoma is a rare occurrence in the setting of a ruptured or leaking silicone breast implant capsule. However, the oncogenic process in such a context is yet another intriguing example of how chronic inflammation plays an integral role in the tumorigenic process. Recent papers have shed light on how inflammation involving M2-tpe macrophages, lymphocytes, and other inflammatory cells at first provide the cytokines necessary to fend off an inciting foreign-body attack such as silicone from a ruptured or leaking implant. Although such a response is beneficial in keeping infection or foreign material in check, there are instances when the response may be exaggerated. It is thought that the extent and length of the response, the balance between pro-inflammatory/anti-apoptotic and anti-inflammatory/pro-apoptotic, as well as genetic factors play important roles in determining the final result of an inflammatory insult.

Serous Papillary Cystadenocarcinoma Arising From Autografted Ovary of the Abdominal Wall

Gong, W., Cheng, H., Yan, Q., Huang, G., Luo, P., Wang, Z. — Wed, 14 Jan 2009 22:31:23 PST

A 58-year-old-woman developed a serous papillary cystadenocarcinoma between the fascia and peritoneum of the left abdominal wall. The patient had undergone bilateral oophorectomy for serous cystadenoma 17 years earlier and her residual normal ovarian parenchyma had also been transplanted to the abdominal wall. Grossly and microscopically, the current tumor arises from the autografted ovarian parenchyma. Literature review indicates that carcinoma arising from autografted ovarian tissue is extremely rare.

Renal Sinus Myelolipoma Coexistent With Renal Pelvis Papillary Transitional Cell Carcinoma: A Case Report

Greaves, W. O., Khanna, P., DeLellis, R., Glasser, L., Wang, L. — Wed, 14 Jan 2009 22:31:22 PST

A unique case of renal sinus myelolipoma presenting as a mass coexistent with papillary transitional cell carcinoma is reported. The patient was a 64-year-old man with a history of bladder transitional cell carcinoma. He presented with gross hematuria and a filling defect in the renal pelvis on computed tomography scan. Pathological findings revealed an irregular myxoid fatty mass, in addition to high-grade papillary transitional cell carcinoma. The differential diagnosis included myxoid liposarcoma, myxoid variant of malignant fibrous histiocytoma (myxofibrosarcoma), and angiomyolipoma. Immunoperoxidase staining confirmed the presence of hematopoietic cells, whereas diagnostic histological and immunohistochemical features of liposarcoma, myxofibrosarcoma, and angiomyolipoma were absent. Myelolipoma and papillary transitional cell carcinoma appear to be unrelated coexistent entities in this case.

Capillary Hemangioma of the Testis

Talmon, G. A., Stanley, S. M., Lager, D. J. — Wed, 14 Jan 2009 22:31:23 PST

Testicular hemangiomas are benign vascular neoplasms usually occurring in children. Pathologists must be aware of this entity when performing intraoperative frozen sections as, after diagnosis, testicle-sparing surgery is adequate. The authors present a typical case with immunohistochemical studies and a review of the literature.

Clinicopathologic Analysis of 4 Perivascular Epithelioid Cell Tumors (PEComas) of the Gastrointestinal Tract

Shi, H.-y., Wei, L.-x., Sun, L., Guo, A.-t. — Sun, 04 Jan 2009 20:28:43 PST

This study describes the clinical, histologic, and immunohistochemical features of four cases of GI PEComa. The size of the tumors ranged from 3.5 to 6.0cm in diameter, and all were located in wall of the large bowel. Microscopically, the tumors were characterized by an epithelioid arrangement of tumor cells, which had abundant clear to pale eosinophilic granular cytoplasm, vesicular nuclei and prominent nucleoli. The stroma was rich in capillaries, a sinusoidal vasculature and thick-walled blood vessels. Mitotic figures were generally rare. Immunohistochemically, the tumors were positive for Vimentin (4/4), HMB-45 (4/4), Melan-A(4/4),alpha-smooth muscle actin (4/4), and desmin (3/4). The patients have been well with no evidence of disease at 8, 15, 32 and 36 months,respectively, after the surgical operation. GI tract PEComas should be regarded as tumors of an uncertain malignant potential until long-term outcome data for a larger number of patients become available.

Basal Cell Carcinoma of the Buccal Mucosa in a Nevoid Basal Cell Carcinoma Syndrome Patient

Shumway, B., Kalmar, J., Rawal, Y., Allen, C. — Sun, 04 Jan 2009 22:57:11 PST

Basal cell carcinoma (BCC) of the oral cavity is a controversial lesion with clinical and histopathologic features that overlap with those of peripheral ameloblastoma (PA). Ber-EP4, a cell surface glycoprotein preferentially expressed in BCC of the skin, has been suggested as a useful marker to support the diagnosis of oral BCC. This study presents a case of intraoral BCC arising in the anterior buccal mucosa of a patient with nevoid basal cell carcinoma syndrome (NBCCS), which represents a previously unreported clinical finding, to our knowledge. Histopathologic and immunohistochemical features of the case were compared to examples of PA, conventional intraosseous ameloblastoma, sporadic cutaneous BCC and cutaneous BCC from NBCCS patients. Ber-EP4 expression by the oral tumor was distinct from both peripheral and intraosseous forms of ameloblastoma and was identical to cutaneous BCC in both sporadic and syndromic settings.

The Histological Features of Intestinal Spirochetosis in a Series of 113 Patients

Carr, N. J., Mahajan, H., Tan, K., Sharma, R. — Tue, 30 Dec 2008 22:44:56 PST

A multicenter retrospective review of cases diagnosed as spirochetosis was performed to determine the presenting symptoms and histological changes. A total of 113 cases were retrieved from the archives, consisting of 97 colorectal specimens and 16 appendices. In only 25 cases was the presenting symptom recorded as diarrhea. Of the colorectal specimens, 87 (90%) showed no mucosal abnormality (apart from the spirochetes); the other 10 showed mucosal inflammation but 6 of them had a diagnosis of another inflammatory disease process accounting for the inflammatory changes. Five appendices showed acute appendicitis; the other 11 were unremarkable. It is concluded that spirochetosis in an unselected general population is unlikely to be of pathological significance. Furthermore, if spirochetosis is observed in an inflamed biopsy, it is most likely to be an incidental finding.

Hepatic Rosai-Dorfman Disease With Coincidental Lymphoma: Report of a Case

Di Tommaso, L., Rahal, D., Bossi, P., Roncalli, M. — Tue, 30 Dec 2008 22:44:57 PST

Rosai–Dorfman disease (RDD; sinus histiocytosis with massive lymphoadenopathy) is a rare, usually nodal self-limited disease. Sometimes nodal disease is coupled with extranodal localizations, in which case the disease can follow a protracted clinical course characterized by remissions and relapses and, exceptionally, a fatal outcome. Exclusive extranodal localizations are rare and their prognosis variable. This study reports a case of RDD apparently limited to the liver coexisting with a diffuse (stage IV) relapsing follicular lymphoma. The patient is alive and well 24 months after the diagnosis of the lymphoma. It is conceivable that the lymphoma has induced RDD via an immunological disorder, possibly involving interleukin expression. The favorable outcome supports the belief that the prognosis of RDD is largely dependent on the number of extranodal sites involved, rather than on the specific sites themselves.

Diffusely Infiltrating Endometrial Carcinomas With No Stromal Response: Report of a Series, Including Cases With Cervical and Ovarian Involvement and Emphasis on the Potential for Misdiagnosis

Kalyanasundaram, K., Ganesan, R., Perunovic, B., McCluggage, G. — Tue, 30 Dec 2008 22:44:56 PST

Endometrial carcinomas, particularly of endometrioid type, can invade the myometrium or cervix without eliciting a stromal desmoplastic or inflammatory response and have been referred to as diffusely infiltrating endometrial carcinomas. This study describes a series of 14 endometrial carcinomas infiltrating as single "naked" glands without a stromal response. The neoplasms consisted of 12 endometrioid carcinomas, 1 mixed endometrioid and clear cell carcinoma, and 1 serous carcinoma. In all cases, there was myometrial invasion without stromal response. Seven cases exhibited cervical stromal involvement and in 2 there was involvement of both ovaries in a similar pattern.Several of the cases were seen in consultation and the pattern of infiltration raised a number of differential diagnoses, both benign and malignant, depending on the site of tumor involvement, including adenomyosis, adenomyoma, primary endocervical glandular lesions, cervical mesonephric remnants, endometriosis or tuboendometrioid metaplasia, and ovarian cortical inclusion cysts. Although this pattern of invasion has been reported previously, it continues to present diagnostic difficulties.

Simultaneous Occurrence of IgG4-Related Chronic Sclerosing Dacryoadenitis and Chronic Sclerosing Sialadenitis in a Patient Also With Involvement of Lymph Node and Warthin's Tumor

Lee, L.-Y., Chen, T.-C., Kuo, T.-t. — Tue, 30 Dec 2008 22:44:55 PST

Chronic sclerosing dacryoadenitis and chronic sclerosing sialadenitis have been shown to belong to the group of diseases referred to as IgG4-related sclerosing disease. The authors report a case of the simultaneous occurrence of IgG4-related sclerosing disease in both lacrimal and submandibular glands, clinically simulating malignant lymphoma. A cervical lymph node and a Warthin’s tumor were also involved. This unique case of multiple organ involvement in IgG4-related sclerosing disease is documented.

Collagenous Gastritis

Jain, R., Chetty, R. — Mon, 22 Dec 2008 01:30:09 PST

A 25-year-old patient presented with epigastric pain, which on gastric biopsy revealed the characteristic appearance of collagenous gastritis. There was a thick prominent subepithelial band that was confirmed to be collagen with a Masson’s trichrome stain. There was associated Helicobacter pylori gastritis but no evidence of a lymphocytic gastritis. The patient did not have watery diarrhea. Collagenous gastritis can occur in young patients, be restricted to the stomach, and can be associated with celiac disease.

Prostate Melanosis Associated With Acinar Adenocarcinoma

Klock, C., Gomes, R., Joao, M., Netto, G. — Thu, 18 Dec 2008 22:31:32 PST

Prostate melanosis is an uncommon lesion of uncertain etiology where melanin deposition is seen in the epithelium, in the stroma, or in the form of a blue nevus. There are three reports in the literature of melamosis seen in association with prostatic adenocarcinoma. This is the fourth report of this association.

Pulmonary Carcinoid Tumor Masquerading as a Synovial Sarcoma

Zhong, Y., Wu, S., Andaz, S., Danovich, S., Lin, J. — Thu, 18 Dec 2008 22:31:32 PST

A rare case of pulmonary carcinoid tumor exhibiting morphologically as a monophasic synovial sarcoma is reported. The patient is a 37-year-old man who presented with shortness of breath for several weeks. The chest computed tomographic scan showed a 3.5 cm pulmonary mass in the left lower lobe. The lesion was an oval, tan nodule displaying features of monophasic synovial sarcoma. Immunohistochemical studies were performed and included a carcinoid tumor as a main differential diagnosis. Reverse transcriptase polymerase chain reaction and fluorescence in situ hybridization studies were negative for a molecular evidence of synovial sarcoma. This case further emphasizes the capacity of pulmonary carcinoid tumor cells to show various morphologic expressions even toward a mesenchymal differentiation mimicking a synovial sarcoma.

Dedifferentiated Chromophobe Renal Cell Carcinoma With Massive Osteosarcoma-Like Divergent Differentiation: A Singular Entity in the Spectrum of Retroperitoneal Calcifying Tumors

Gira, F., Barbieri, A., Fellegara, G., Zompatori, M., Corradi, D. — Thu, 18 Dec 2008 22:31:32 PST

Sarcomatoid change in renal cell carcinoma is the result of the dedifferentiation of the "parent" tumor into a high-grade malignancy characterized by sarcoma-like features and associated with an accelerated clinical course and poorer prognosis. Any of the renal cell carcinoma subtypes can undergo sarcomatoid dedifferentiation, with the chromophobe variant being the most prone. The present report describes the case of a woman affected by a classic chromophobe renal cell carcinoma that developed dedifferentiation accompanied by a very rare osteosarcoma-like divergent differentiation, which constituted about 90% of the entire retroperitoneal mass. In addition to presenting the relevant imaging, histopathological, and immunohistochemical findings, this article briefly discusses the main differential diagnosis of retroperitoneal ossifying/ calcifying masses, which could give rise to diagnostic problems either in small biopsies or at imaging.

Juxtaglomerular Cell Tumor of the Kidney: Case Report and Differential Diagnosis With Emphasis on Pathologic and Cytopathologic Features

Mendez, G. P., Klock, C., Nose, V. — Thu, 18 Dec 2008 22:31:31 PST

This study presents a case of juxtaglomerular cell tumor (JCT) in a 12-year-old girl with hypertension. Fine needle aspirate (FNA) cytology demonstrated a neoplasm with features of a papillary carcinoma, prompting a right radical nephrectomy. Histological examination revealed solid sheets of round epithelioid cells with eosinophilic granular cytoplasm, and distinct cell borders in a background of widespread hemorrhage. Electron microscopy revealed cytoplasmic renin granules. The differential diagnosis of a renal mass in a young patient with hypertension includes JCT, Wilm’s tumor, and renal cell carcinoma, which may produce renin. The renin granules detected by electron microscopy are characteristic of JCT, and the diagnosis is confirmed by ultrastructural study. FNA cytology is not sensitive enough for the diagnosis of JCT and its results must be carefully interpreted.

Benign Spindle Cell Tumor Not Otherwise Specified (NOS) in a Male Breast

Mon, 08 Dec 2008 03:21:40 PST

Breast spindle cell tumors (BSCTs), although uncommon, constitute a heterogeneous group of benign and malignant lesions, often necessitating different therapeutic approaches. This study describes the case of a 62-year-old man who displayed a gradually growing retroareolar tumor of the left breast. The mass was well circumscribed, unilateral, and grossly nodular. The patient eventually underwent wide local excision of the mass. The lesion was made up of spindle cells arranged in fascicular clusters, separated by bands of collagen. No mitotic figures were observed. Immunohistochemically, the mass expressed strong and diffuse cytoplasmic staining for vimentin, CD34, CD10, and bcl-2, whereas it was negative for cytokeratins, smooth muscle actin, desmin, S-100 protein, p53, Ki-67, estrogen and progesterone receptors. Diverse histological results and immunohistochemical features established the diagnosis of benign BSCT, not otherwise specified. The patient remains disease-free 12 months after lumpectomy. This case report adds to the spectrum of the benign BSCTs and delineates the nature of different types of these lesions, in order to carefully select optimal therapeutic regimes.

HIV-Unrelated Benign Lymphoepithelial Cyst of the Parotid Glands Containing Lymphoepithelial Lesion-Like Structures: A Report of 3 Cases

Kojima, M., Nakamura, N., Matsuda, H., Kaba, S., Itoh, H., Masawa, N. — Mon, 08 Dec 2008 03:21:40 PST

Benign lymphoepithelial cyst (BLC) of the salivary gland is uncommon. Among report of these lesions, there were no previous cases of lymphoepithelial lesion (LeL) in human immunodeficiency virus type-1 (HIV-1) unrelated BLC. This study reports 3 cases of HIV-unrelated BLC containing LeL-like structures. Pathologically, 3 lesions were characterized by cuboidal epithelial-lined, cystic spaces, numerous lymphoid follicles, and dense lymphoplasmacytoid infiltrate. Moreover, small lymphocytes invaded the epithelial clusters forming LeL-like structures. Histological findings of 3 lesions resembled marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. However, staining with CD3, CD5, and CD20 demonstrated the mixed nature of small lymphocytes invading the epithelial clusters (LeLlike structures). Moreover, there were no CD43+ B-lymphocytes in any of the cases. The polytypic nature of B-lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction. The 3 cases indicate that HIV-unrelated BLC should be added to the differential diagnosis of MALT-type lymphoma involving the parotid glands.

Oral Hairy Leukoplakia in HIV-Negative Patients: Report of 10 Cases

Piperi, E., Omlie, J., Pambuccian, S., Koutlas, I. G. — Tue, 25 Nov 2008 02:51:07 PST

Ten cases of oral hairy leukoplakia (OHL) in HIV-negative patients are presented. Eight of the 10 patients were on steroid treatment for chronic obstructive pulmonary disease, 1 patient was on prednisone as part of a therapeutic regimen for gastrointestinal stromal tumor, and 1 patient did not have any history of immunosuppression. There were 5 men and 5 women, ages 32-79, with mean age being 61.8 years. Nine out of 10 lesions were located unilaterally on the tongue, whereas 1 lesion was located at the junction of the hard and soft palate. All lesions were described as painless, corrugated, nonremovable white plaques (leukoplakias). Histologic features were consistent with Epstein–Barr virus–associated hyperkeratosis suggestive of OHL, and confirmatory in situ hybridization was performed in all cases. Candida hyphae and spores were present in 8 cases. Pathologists should be aware of OHL presenting not only in HIV-positive and HIV-negative organ transplant recipients but also in patients receiving steroid treatment, and more important, certain histologic features should raise suspicion for such diagnosis without prior knowledge of immunosuppression.

Sclerosing Spindle Cell Rhabdomyosarcoma in an Adult: Report of a New Case and Review of the Literature

Gavino, A. C. P., Spears, M. D., Peng, Y. — Mon, 17 Nov 2008 21:25:25 PST

Sclerosing spindle cell rhabdomyosarcoma (SSRMS) is a newly recognized entity in adults. The authors report a new case of SSRMS in a 31-year-old woman who presented with a large right leg mass. Biopsy revealed a malignant spindle cell neoplasm with focal sclerotic areas. A diagnosis of monophasic synovial sarcoma was favored initially. The tumor cells in the resection specimen were positive for myosin, myogenin, and MyoD1. Fluorescence in situ hybridization performed on the resection specimen showed no evidence of SYT gene rearrangement in the neoplastic cells, ruling out monophasic synovial sarcoma. A diagnosis of SSRMS was established. The patient succumbed to widely metastatic disease 16 months after initial diagnosis. This case highlights the utility of skeletal muscle markers and cytogenetic testing in distinguishing SSRMS from its mimic, monophasic synovial sarcoma. It is hoped that this case will expand the literature on adult SSRMS and help clinicians and pathologists better understand this newly described entity.

Osteosarcoma Arising in a Long-Standing Uterine Leiomyoma: A Case Report and Literature Review

Wang, R. C., Wen, M. C., Wang, J., Ho, S. C., Jan, Y. J. — Mon, 17 Nov 2008 21:25:25 PST

The authors present the first case of a pure osteosarcoma arising in a long-standing uterine leiomyoma along with a literature review of all heterologous sarcomas arising in uterine leiomyoma. Most cases present with abnormal vaginal bleeding and symptoms related to a rapidly enlarged pelvic mass in postmenopausal women with a long-standing history of uterine leiomyoma. The histological finding of the case in this study revealed a relatively well-circumscribed tumor with a peripheral ring of leiomyoma and a central osteosarcoma. The case in this study further supported a possible pathogenesis involving the p53 gene. The prognosis may be better with low-stage tumor; however, cases with advanced stages, larger tumor size at presentation, and histological type of epithelioid angiosarcoma had poorer prognosis despite aggressive therapy.

Pulmonary Talc Granulomatosis Masquerading as Massive Pulmonary Embolism

Agarwal, A. M., Grey, T. — Wed, 22 Oct 2008 00:43:34 PDT

Small-Cell Neuroendocrine Carcinoma of the Nasopharynx: Report of a Rare Case Lacking Association With Epstein-Barr Virus

Lee, L.-y., Chang, K.-p., Hsu, C.-L., Chen, T.-C., Kuo, T.-t. — Sun, 12 Oct 2008 21:18:18 PDT

Only 2 cases of nasopharyngeal small cell carcinoma have been reported. Another case of this rare type of nasopharyngeal tumor confirmed by immunopositivity for CD56, synaptophysin, and chromogranin A is described in this study. The tumor was also negative for creatine kinase 20 and thyroid transcription factor-1. Clinical investigation did not find any primary tumor in other anatomic sites. Therefore, this case was established as a primary nasopharyngeal small cell carcinoma. Epstein-Barr virus was shown to be absent by Epstein-Barr virus encoded RNA in situ hybridization study in this case, which further distinguished it from conventional nasopharyngeal carcinoma. Clinical features of this case and 2 previously reported cases are compared. They do not differ from conventional nasopharyngeal carcinoma in clinical presentation, but the prognosis seems to be worse. More cases are needed to confirm the observation and for understanding the clinical behavior of this rare type of nasopharyngeal tumor.

B-Cell Cutaneous Lymphoid Hyperplasia Representing Progressive Transformation of Germinal Center: A Report of 2 Cases

Kojima, M., Sakurai, S., Shimizu, K., Itoh, H. — Wed, 24 Sep 2008 06:44:24 PDT

Cutaneous lymphoid hyperplasia (CLH) is a reactive polyclonal benign lymphoproliferative process predominantly composed of B cells or T cells, either localized or disseminated. The authors report histomorphologic, immunophenotypic, and genotypic findings of 2 cases of B-cell CLH demonstrating progressive transformation of germinal center (PTGC). Histologically, most of the lymphoid follicles were PTGCs with a few hyperplastic germinal centers. PTGC was characterized by enlarged but well-circumscribed follicles without clear demarcation of the germinal center and mantle zone, which contained a predominance of small lymphocytes and variable numbers of centrocytes, centroblasts, and immunoblasts. However, there were no centroblasts and immunoblasts resembling lymphocytic and/or histiocytic Reed–Sternberg cell variants in nodular lymphocyte–predominant Hodgkin lymphoma (NLPHL) in either lesion. These unusual CLHs should be differentiated from the primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicular lymphoma, particularly "floral variant," or NLPHL. To avoid over-diagnosis and overtreatment, immunophenotypic and genotypic studies are required along with careful morphologic examination.

Extensive Ganglioneuromatosis of Gallbladder

Sakuma, T., Hirota, M., Ohashi, H., Kakudo, K., Kawano, K. — Fri, 19 Sep 2008 03:40:42 PDT

A case of extensive ganglioneuromatosis (GN) of gallbladder is presented. A 38-year-old man presented with a hepatic hilar mass (~48 mm) and gall stones. He had undergone total thyroidectomy for medullary thyroid carcinoma 8 years earlier. The hepatic hilus tumor, which had been clinically suspected to be a lymph node metastasis from the medullary thyroid carcinoma, was found to be pheochromocytoma. The gallbladder, resected with a clinical diagnosis of cholelithiasis, showed extensive transmural GN despite a grossly normal appearance. Taking into account the past history, the patient was diagnosed as having multiple endocrine neoplasia 2b (MEN2b)–associated GN of gallbladder and ectopic pheochromocytoma. As GN of gallbladder in MEN2b has been rarely reported, the histological findings are described in detail and a brief review of literature is carried out.

Peripheral Eosinophilia Camouflaging Anaplastic Large Cell Lymphoma

Powers, M. L., Watson, B., Frater, J., Kreisel, F., Hassan, A. — Fri, 19 Sep 2008 03:40:41 PDT

Eosinophilia is a nonspecific laboratory finding, often noted incidentally during routine blood analysis. When persistent, eosinophilia can herald an underlying parasitic infection, drug reaction or less commonly, a neoplastic process. Anaplastic large cell lymphoma (ALCL) and tissue eosinophilia has been described; however, such cases have not displayed marked leukocytosis with eosinophilia. This article reports a patient presenting with marked leukocytosis with profound peripheral eosinophilia initially thought to be related to a chronic myeloproliferative disorder, likely chronic eosinophilic leukemia. After further diagnostic evaluation, ALCL was noted in the bone marrow, masked by the myeloid hyperplasia and eosinophilia. This case emphasizes the importance of a full diagnostic workup for T-cell malignancies, including ALCL rather than focusing on the far less common eosinophilia-associated myeloid malignancies in the clinicopathologic setting of marked eosinophilia. Moreover, bone marrow involvement by ALCL is exceedingly rare and when noted, presents as one or more localized lytic lesions. This is the first reported case of ALCL primarily involving bone marrow without radiographic evidence of lytic bone lesions.

Papillary Hemangiomas and Glomeruloid Hemangiomas Are Distinct Clinicopathological Entities

Suurmeijer, A. J. — Fri, 19 Sep 2008 03:40:42 PDT

The author reviews and compares the clinicopathological features of papillary hemangiomas and glomeruloid hemangiomas, 2 rare, cutaneous intravascular capillary-type vascular lesions with overlapping morphological details. Immunostaining for collagen IV highlighted discriminating features in these lesions. Thin basement membranes and glomeruloid architecture are typical of glomeruloid hemangiomas, whereas papillae with thick mantles of a basement membrane–like matrix enveloping pericytes are prominent in papillary hemangiomas. Thus, collagen IV staining patterns provide further evidence that papillary and glomeruloid hemangiomas represent distinct histopathological entities. This additional technique should allow pathologists to readily distinguish between the lesions and make a proper diagnosis. What is important is that glomeruloid hemangiomas—often presenting in a spectrum of multiple cutaneous vascular lesions, including cherry hemangiomas—are a hallmark of POEMS (acronym for polyneuropathy, oganomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome and/or multicentric Castleman’s disease, whereas papillary hemangiomas clinically present as innocent solitary cutaneous hemangiomas in otherwise healthy individuals.

Primary Intratesticular Spindle Cell Tumors: Interdigitating Dendritic Cell Tumor and Inflammatory Myofibroblastic Tumor

Nistal, M., Gonzalez-Peramato, P., Serrano, A., Reyes-Mugica, M., Cajaiba, M. — Fri, 19 Sep 2008 03:40:42 PDT

Spindle cell neoplasms arising in the testis are uncommon; most cases belong to the category of gonadal stromal tumors, and the presence of distinctive clinical and pathological features usually lead to a definitive diagnosis. In some instances, however, the diagnosis of these tumors can be challenging and special techniques are needed. The present study reports 2 unusual cases of primary intratesticular tumors showing a striking morphology, characterized by spindle to stellate cells in a lymphoid background. The diagnosis of interdigitating dendritic cell tumor and inflammatory myofibroblastic tumor was established in the 2 cases, and their probable origin in cells of the accessory immune system is discussed. Although both tumors share similar histological features, their immunohistochemical profiles were decisive for a definitive diagnosis.

Metastasis of Malignant Peritoneal Epithelioid Mesothelioma in Endoscopic Gastric Biopsy: A Diagnostic Pitfall

Daniele, L., Righi, A., Ferraris, A., Macri, L., Asioli, S. — Sun, 14 Sep 2008 22:45:36 PDT

Aim To describe a case of metastasis of malignant peritoneal epithelioid mesothelioma in gastric antral mucosa in a patient with a cryptogenic liver cirrhosis associated with esophageal varices, abdominal pain and distension, ascites, and weight loss. Materials and method The patient underwent esophageal gastric endoscopy for varices, and a biopsy of a polypoid antral lesion was performed. The latter revealed a proliferation of polygonal cells with moderately atypical nuclei and pale eosinophilic, peripherally condensed cytoplasm infiltrating into the lamina propria between the normal mucosal glands of the antrum. The tumor cells were diffusely positive to anticalretinin antibody, whereas anti–claudin 4 and anti-CEA antisera were negative. Conclusions Metastases of malignant peritoneal mesotheliomas are unusual, and a predominantly gastrointestinal localization is rare. Pathologists should be aware of this possibility to avoid misdiagnosis, particularly in small biopsy specimens.

ALK-Positive Anaplastic Large Cell Lymphoma in a Patient With Chronic Lymphocytic Leukemia

Liu, T., He, M., Carlson, D. L., Hedvat, C., Teruya-Feldstein, J. — Sun, 14 Sep 2008 22:45:36 PDT

This article reports the case of a 59-year-old patient with an 8-year history of chronic lymphocytic leukemia (CLL), prostate carcinoma, and squamous cell carcinoma who developed an ALK-positive anaplastic large cell lymphoma (ALCL). Lymph node and bone marrow biopsies showed 2 distinct morphologic populations: (a) the CLL component showing a diffuse monomorphous infiltrate of small lymphocytes with the typical immunophenotype showing positive CD20, CD5, CD23, and light chain restriction and (b) the ALCL component showing large anaplastic pleomorphic cells positive for CD30, CD45, ALK, CD45Ro, CD4, and vimentin. Polymerase chain reaction performed on the lymph node for immunoglobulin heavy chain and T-cell receptor and β showed gene rearrangements after macrodissection of morphologically distinct populations, indicating confirmed genetically distinct populations. Despite intensive chemotherapy, the patient died. This case represents the rare occurrence of an ALK-positive ALCL developing in a patient with CLL.

PUNLMPs are Sunflowers and Not Snowglobes

Dalla Palma, P. — Sun, 14 Sep 2008 22:45:36 PDT

Prostate Cancer Presenting as an Endobronchial Mass: A Case Report With Literature Review

Garai, S., Pandey, U. — Sun, 14 Sep 2008 22:45:35 PDT

Endobronchial metastasis from a prostate cancer is uncommon. Diagnosis of prostate carcinoma after primary presentation with an endobronchial mass is very rare. The authors describe the case of an 84-year-old man with endobronchial metastases from prostatic carcinoma presenting primarily with pulmonary symptoms. The diagnosis of prostate cancer and endobronchial metastases was made by a bronchoscopic bronchial biopsy and confirmed by immunohistological staining with prostate-specific antigen. The importance of this manifestation along with clinical and therapeutic implications is discussed here.

Ovarian Malignant Melanoma: A Clinicopathologic Study of 5 Cases

Ueng, S.-H., Pinto, M., Alvarado-Cabrero, I., Lee, L.-Y., Tavassoli, F. — Sun, 14 Sep 2008 22:45:36 PDT

Primary ovarian malignant melanomas are extremely rare. The wide range of morphologic appearances assumed by melanomas in the ovary can cause considerable difficulty in diagnosis. The clinicopathologic features of 4 definite and 1 probable primary ovarian melanomas are presented. The patients ranged in age from 41 to 71 years. Four tumors were within teratomas with 2 showing a lentiginous pattern of melanoma in the squamous epithelium. Unusual histologic features were noted. Immunostains for S-100, HMB-45, and Melan-A were positive in all tumors. Premelanosomes were identified in 2 tumors ultrastructurally. Metastatic sites included regional nodes, peritoneal surfaces, omentum, lung, liver, brain, and bone. All 5 patients died within 18 months. Immunohistochemistry and electron microscopy aid considerably in the diagnosis of ovarian melanomas where pigmentation or teratomatous elements are absent. Familiarity with the wide range of morphologic patterns presented here will raise awareness and facilitate detection of future cases of ovarian melanoma.

Cytomegalovirus Prostatitis: A Series of 4 Cases

Yoon, G.-S., Nagar, M. S., Tavora, F., Epstein, J. I. — Tue, 12 Aug 2008 22:51:27 PDT

Cytomegalovirus (CMV) prostatitis is very rare with only 1 report of biopsy-proven CMV prostatitis in the literature. The authors report 4 cases, 3 detected on needle biopsy and 1 detected on transurethral resection. Patients were 36, 41, 48, and 71 years old. All patients were immunosuppressed, including 1 with AIDS and 3 undergoing immunosuppressive therapy following organ transplantation. CMV inclusions were seen in secretory cells of the prostatic glands, endothelial cells of small vessels, and prostatic stromal cells associated with a dense lymphoid inflammation. Only very rarely is CMV prostatitis detected on clinical specimens, typically in immunosuppressed hosts undergoing immunosuppressive therapy following organ transplantation. Patients with CMV prostatitis may harbor multiple infections or have other serious medical conditions adversely affecting their prognosis.

Pulmonary Sequestration With CCAM Type II Appearing as Adrenal Tumor Protruding Through a Bochdalek Hernia

Tue, 12 Aug 2008 22:51:27 PDT

A case of a pulmonary sequestration, which almost exclusively consisted of a congenital cystic adenomatoid malformation type II located subdiaphragmatically in the left retroperitoneal area, is reported. This case, in a 24-year-old male patient, is unique in that it appeared as an adrenal incidentaloma and extended through a Bochdalek hernia into the pleural space. It was discovered upon routine ultrasound screening for hepatocellular carcinoma in a patient with a carrier state for hepatitis B. Diagnosis was established only upon histological analysis of the surgically removed tumor after staining with hematoxylin and eosin as well as surfactant A and B. The location of the tumor may indicate that it was formed by an entrapment of a lung bud by the developing diaphragm. This appearance may give us insight into the formation of such tumors. It also highlights the difficulty of diagnosing subdiaphragmatic retroperitoneal tumors without histological examination.

Massive Localized Lymphedema With Unusual Presentations : Report of Two Cases and Review of the Literature

Bogusz, A. M., Hussey, S. M., Kapur, P., Peng, Y., Gokaslan, S. T. — Tue, 08 Jul 2008 01:24:34 PDT

Massive localized lymphedema is a benign soft tissue lesion that usually presents as a large mass in morbidly obese adults. The diagnosis may be challenging as it can mimic other lesions, including well-differentiated liposarcoma. We report 2 cases of massive localized lymphedema with unusual presentation. The first case is a recurrent massive localized lymphedema in the right thigh of a 40-year-old morbidly obese woman. In addition to typical massive localized lymphedema features such as prominent edema and vascular proliferation in the adipose tissue, we observed prominent and abundant multinucleated stromal floret-like giant cells, arborizing network of capillaries, and areas of hyalinized collagen. Our second case is in a rare location (scrotum extending into penile soft tissue) in an overweight 55-year-old male. This lesion exhibits striking smooth muscle hyperplasia. Lack of staining by antibodies against murine double minute 2 protein and cyclin dependent kinase 4 and absence of high mobility group AT- hook 2 transcription factor rearrangement by fluorescence in situ hybridization support our diagnosis of massive localized lymphedema in both cases.

E-selectin and Sialyl Lewis X Expression Is Associated With Lymph Node Metastasis of Invasive Micropapillary Carcinoma of the Breast

Wei, J., Cui, L., Liu, F., Fan, Y., Lang, R., Gu, F., Guo, X., Tang, P., Fu, L. — Tue, 08 Jul 2008 01:24:34 PDT

To investigate the possible roles of E-selectin and its ligand, Sialyl Lewis X, in lymph node metastasis of invasive micropapillary carcinoma of the breast, 100 cases of invasive micropapillary carcinoma and 97 cases of invasive ductal carcinoma were analyzed immunohistochemically for the expression of E-selectin and Sialyl Lewis X, along with CD34, to measure the microvessel density of invasive micropapillary carcinoma. We found that the number of E-selectin–positive vessels was greater in invasive micropapillary carcinoma than in invasive ductal carcinoma, and it was significantly correlated with the histological grade, the number of positive lymph nodes, and the microvessel density of invasive micropapillary carcinoma. The Sialyl Lewis X expression of invasive micropapillary carcinoma was higher than that of invasive ductal carcinoma, which was also associated with lymph node metastasis. In invasive micropapillary carcinoma, the Sialyl Lewis X expression was predominantly in the stroma-facing surface of the cell clusters and the adjacent stroma, while in invasive ductal carcinoma it was largely intracytoplasmic or intercellular. These findings suggested that E-selectin and Sialyl Lewis X might play an important role in lymph node metastasis in invasive micropapillary carcinoma. The expression pattern of Sialyl Lewis X in invasive micropapillary carcinoma suggested that the reversal of cell polarity of invasive micropapillary carcinoma might be as an important factor for the morphogenesis and possibly the pathogenesis, especially their higher rates of lymph node metastasis.

Pseudoangiomatous Stromal Hyperplasia (PASH) of the Breast With Foci of Morphologic Malignancy: A Case of PASH With Malignant Transformation?

Nassar, H., Elieff, M. P., Kronz, J. D., Argani, P. — Tue, 08 Jul 2008 01:24:32 PDT

Pseudoangiomatous stromal hyperplasia (PASH) is a benign proliferation of the hormonally responsive, specialized mammary stroma characterized by slit-like pseudovascular spaces lined by bland spindle cells. It is usually an incidental microscopic finding but in some cases it may present as a slowly growing mass. A malignant counterpart for this lesion has not been reported. We describe a case of PASH with foci of malignant histologic features presenting as a slowly growing mass in a 30-year-old woman. The previously reported variants of PASH and the other mammary stromal lesions related to PASH are also discussed. This is perhaps the first case of PASH with foci of malignant histologic features reported in the literature and represents a rare sarcoma derived from specialized hormonally responsive mammary stroma.

Histopathological Aspects of Neurocryptococcosis in HIV-Infected Patients: Autopsy Report of 45 Patients

Klock, C., Cerski, M., Goldani, L. Z. — Tue, 08 Jul 2008 01:24:34 PDT

The authors describe the histopathological necropsy findings of 45 human immunodeficiency virus (HIV)–infected patients with neurocryptococcosis. Systemic cryptococcosis with involvement of multiple organs such as spleen, liver, and lungs was present in all patients. Predominant diffuse meningoencephalitis predominantly in the basal ganglia, thalamus, and midbrain, with minimal inflammatory infiltrate was seen in 30 AIDS patients (70%). We frequently observed in those patients the presence of multiple gelatinous pseudocysts with abundant Cryptococcus neoformans in the Virchow-Robin spaces and adjacent brain caused by the dissemination of the meningeal infection along the perivascular spaces. Isolated meningeal and cerebral involvement with minimal inflammatory infiltrate with numerous fungal organisms, and occasionally with granulomatous reaction and necrosis, was seen in 15 patients (30%). In addition to involvement of multiple organs by C. neoformans, HIV-infected patients with clinical manifestations of neurocryptococcosis frequently present a widespread involvement of the brain.

Primary Capillary Hemangioblastoma of Bone: Report of a Case Arising in the Sacrum

Panelos, J., Beltrami, G., Capanna, R., Franchi, A. — Tue, 08 Jul 2008 01:24:33 PDT

Capillary hemangioblastoma (CHB) is a benign, highly vascularized tumor that generally occurs in the central nervous system either in the setting of von Hippel-Lindau (VHL) disease or, more often, as a solitary sporadic lesion that is increasingly recognized in extraneural sites. We present the case of a 72-year-old woman with low back pain and a well-demarcated lytic lesion of the sacrum, which at histological and ultrastructural examination was indistinguishable from central nervous system CHB. The patient had no signs of VHL disease and died of another cause with no evidence of disease 57 months after curettage of the lesion. To our knowledge, this is the second case of CHB reported to occur in bone.

IgG4-Positive Sclerosing Cholangitis Following Autoimmune Pancreatitis With Deranged CA19.9

Tue, 08 Jul 2008 01:24:33 PDT

Sclerosing cholangitis is an autoimmune condition characterized by lymphocytic infiltration within the biliary epithelium leading to multifocal stricturing of the biliary tree. Primary sclerosing cholangitis (PSC) is the most common type encountered clinically. However, a similar process may occur in conjunction with autoimmune pancreatitis (AIP), known as AIP-associated sclerosing cholangitis (AIP-SC). This subtype is associated with an elevated IgG₄ level and the presence of a number of autoantibodies. AIP-SC shows good response to steroid treatment, distinguishing it clinically from PSC. The authors report a case of AIP-SC in a patient who had previously undergone a biliary bypass for AIP-induced chronic pancreatitis. The presentation of jaundice and grossly elevated tumor marker, CA19.9, raised the concern of malignancy. The uncertainty of the diagnosis was resolved when AIP-SC was confirmed on liver biopsy, with a concomitantly elevated serum IgG₄ level. The disease went into remission with steroid treatment.

Papillary Adenocarcinoma of the Thymus: Case Report and Review of the Literature

Furtado, A., Nogueira, R., Ferreira, D., Tente, D., Eisele, R., Parente, B. — Tue, 08 Jul 2008 01:24:31 PDT

A 44-year-old male with a mediastinal mass measuring 3.5 x 3.5 x 3 cm was diagnosed with papillary adenocarcinoma of the thymus. Other origins of papillary adenocarcinoma were excluded by clinical, imaging, and immunocytochemical methods before assuming this diagnosis. Residual thymus was seen under the microscope. Focal CD5 immunoreactivity was present. There was no associated thymoma. The patient underwent surgery, radiotherapy, and chemotherapy. He disclosed systemic recurrence at 18 months (subcutaneous nodule). He is alive after 24 months of follow-up with active disease. There had been only 7 cases of this rare entity published before.

Incidence of Melanocytic Lesions of the Conjunctiva in a Review of 10 675 Ophthalmic Specimens

Tue, 08 Jul 2008 01:24:30 PDT

During the study period, 10 675 human ophthalmic specimens were received at The Henry C. Witelson Ophthalmic Pathology Laboratory and Registry, McGill University, Montreal, Canada. Of those, 271 were conjunctival lesions (2.5%), with 101 being classified as melanocytic: 50 (49.5%) nevi, 36 (35.6%) primary acquired melanoses, and 15 (14.9%) melanomas. After exclusion of referred cases, 85 lesions were included in the study: 44 (51.7%) nevi, 33 (38.8%) primary acquired melanoses, and 8 (9.4%) melanomas. The most prevalent location was the bulbar conjunctiva. Conjunctival melanomas were most commonly found in an older age group than primary acquired melanosis or nevi. Conjunctival nevi were subdivided into compound (32.9%), subepithelial (16.4%), and junctional (2.3%). Primary acquired melanosis were further classified into primary acquired melanosis with atypia (8.2%) and primary acquired melanosis without atypia (30.5%). Primary acquired melanoses was the predisposing lesion in 75% of the cases of melanoma. In our sample, referral bias could alter the distribution of conjunctival pigmented lesions, with a shift toward the malignant end.

Aggressive Osteoblastoma: A Case Report Involving a Unique Chromosomal Aberration

Tue, 08 Jul 2008 01:24:32 PDT

Osteoblastomas are rare bone-producing neoplasms that generally occur in the young and can be misdiagnosed as an osteosarcoma if correlation with clinical history, radiology, and histology is not carefully considered or if the several variants of osteoblastoma are not recognized. These variants lie on a morphologic spectrum between conventional osteoblastoma and osteosarcoma. Aggressive osteoblastoma is one such subtype. As the name implies, the histologic features of aggressive osteoblastoma may appear malignant, and its biologic behavior may separate it from conventional osteoblastoma. We report a case of aggressive osteoblastoma occurring in the femoral diaphysis of a 12-year-old girl; this osetoblastoma was dyssynchronous from the radiologic appearance and a diagnostic challenge. Cytogenetic evaluation of the neoplasm revealed a pseudodiploid clone with a balanced translocation involving chromosomes 4, 7, and 14. Using the premise that cytogenetics might be useful as a diagnostic tool for a more specific classification, we reviewed the literature in order to compare our findings with known chromosomal aberrations.

Myositis Ossificans Associated With Subclinical Idiopathic Thrombocytopenic Purpura: Report of a Case

Onoda, N., Yamazoe, S., Tanaka, T., Ishikawa, T., Wakasa, K., Hirakawa, K. — Tue, 08 Jul 2008 01:24:31 PDT

A 56-year-old healthy man noticed a stony-hard mass gradually grown in his neck for more than a 6-year period. Examinations revealed 2 additional calcified masses at the esophageal hiatus and retroperitoneum. The cut-surface of the operative specimens showed fibrous and mucinous appearance with many calcified islands. Microscopically, the lesion showed a gradual transform from the central immature area with fibroblasts and mucinous matrix, to the peripheral mature area with woven bone and osteoclasts (zoning phenomenon), which was compatible with the features of mature myositis ossificans. Myositis ossificans is an isolated, benign, nonneoplastic disease typically found in adolescence, after traumatic episode. Similar lesions have been found in the major musculature of patients with increased bleeding tendency. In the present case, idiopathic thrombocytopenic purpura was found to be associated and was suggested to be involved in initiating myositis ossificans. Still, it is extremely rare to observe lesions in these sites synchronously.

Adenocarcinoma Arising in an Elderly Patient""s Large Ileal Duplication

Tue, 08 Jul 2008 01:24:30 PDT

Bowel duplications are rare congenital anomalies commonly found in pediatric patients; few cases may remain undetected until adulthood. Malignant carcinomatous changes are rare complications in intestinal duplications. An 88-year-old female patient was referred to our surgical unit with the diagnosis of a large abdominal mass. An explorative laparotomy was performed, revealing a large (22 x 11 cm) neoplasm strictly connected to the lowest ileal segment and completely filling the pelvis. Definitive histology revealed a moderately differentiated adenocarcinoma developing in a duplication of the terminal ileum. The hypothesis of a gastrointestinal duplication should be evaluated in the differential diagnosis of large, complex, indeterminate masses located in or near the bowel; the possibility of neoplasm within the duplication should be considered.

Clear Cell Change in Colon Polyps

Tue, 08 Jul 2008 01:24:30 PDT

In this study, the pathogenesis of clear cell change in colorectal epithelial lesions was studied. A total of 4 cases of clear cell change (1 hyperplasic polyp and 3 adenomas) were characterized using histochemistry, immunohistochemistry, and electron microscopy. All lesions developed in the left colon. In all, 1 adenoma with clear cell dysplastic glands progressed to adenocarcinoma without clear cell change. Clear cell cytoplasmatic vacuoles were negative for glycogen and mucins (MUC 2, MUC 5AC). Ki-67 LI in clear cell adenoma components was lower than in common adenoma components of the same dysplasia grades (while p53 and β-catenin were similarly expressed). Ultrastructural features of clear cell change showed features of lipid-like material. Clear cell change is found in hyperplastic and neoplastic lesions of the colon and is not due to the accumulation of glycogen or mucins. A degenerative nature of clear cell change is suggested by the demonstration of lipid-like material in the vacuoles of clear cells.

Malakoplakia of the Spleen: A Case Report

Greaves, W. O., Wang, L. J. — Wed, 28 May 2008 02:41:56 PDT

Malakoplakia is an uncommon chronic inflammatory disorder, which is characterized by the presence of histiocytes containing concentric concretions known as Michaelis-Gutmann bodies in a background of mixed inflammation. The urinary tract is the most commonly involved site. However, malakoplakia can be found in a wide range of other organs throughout the body. Its occurrence has been attributed to a defect in the bactericidal capacity of phagocytic cells, and it is usually seen in patients with some degree of immunologic compromise. A case of malakoplakia of the spleen in a patient with Crohn’s disease is reported in this article.

Histopathological Diagnosis of Intestinal Spirochetosis in a Nonimmunocompromised Patient

Suarez-Penaranda, J. M., Fernando, M.-G., Jose, L., Forteza, J. — Wed, 28 May 2008 02:41:55 PDT

Metastatic Hepatocellular Carcinoma of Skin Diagnosed With Hepatocyte Paraffin 1 and a-Fetoprotein Immunostainings

Terada, T., Sugiura, M. — Wed, 21 May 2008 22:19:44 PDT

A rare case of skin metastasis of hepatocellular carcinoma diagnosed with an aid of immunohistochemical stainings of hepatocyte paraffin 1 and a-fetoprotein is reported in this study. An 86-year-old Japanese man was admitted to our hospital due to cutaneous mass in the right chest. An incisional biopsy was performed, which showed proliferation of malignant cells with eosinophilic or clear cytoplasm arranged in solid nests. No trabecular pattern was recognized. Sebaceous carcinoma, clear cell sarcoma, malignant granular cell tumor, metastatic renal cell carcinoma, and metastatic hepatocellular carcinoma were suspected on hematoxylin and eosin preparations. An immunohistochemical study showed that the tumor cells were positive for cytokeratins, hepatocyte paraffin 1, and a-fetoprotein but negative for vimentin, desmin, a-smooth muscle actin, S-100 protein, epithelial membrane antigen, carcinoembryonic antigen, chromogranin, neuron-specific enolase, CD10, CD30, CD34, CD45, CD68, and HMB45. Metastatic hepatocellular carcinoma of the skin was diagnosed pathologically. This case suggests that skin tumors with eosinophilic cytoplasm should be examined by hepatocyte paraffin 1 and a-fetoprotein.

Calcifying Telangiectatic Cutaneous Breast Carcinoma Metastasis

Das, P., Ahuja, A., gupta, S. D. — Wed, 21 May 2008 22:19:42 PDT

Ewing Sarcoma and Primitive Neuroectodermal Tumor of the Esophagus: Report of a Case and Review of Literature

Johnson, A. D., Pambuccian, S. E., Andrade, R. S., Dolan, M. M., Aslan, D. L. — Wed, 21 May 2008 22:19:44 PDT

This study presents a case of Ewing sarcoma and primitive neuroectodermal tumor arising in the esophagus of a 44-year-old woman who presented with progressive dysphagia. Imaging studies demonstrated a polypoid lesion in the esophagus. The tumor was characterized by corded and pseudopapillary architecture, cytologic monotony, and low proliferative activity. Immunohistochemical stains were positive for CD99, neuron-specific enolase, vimentin, cyclin D1, p53, and FLI1 gene product. Fluorescence in situ hybridization demonstrated a 22q12 translocation, associated with primitive neuroectodermal tumor in the tumor cells, whereas reverse transcription polymerase chain reaction conformed expression of Ewing sarcoma/FLI1 fusion transcript in the patient’s bone marrow aspirate. Although this is a rare site for this type of tumor to occur, primitive neuroectodermal tumor should be considered in the differential diagnosis of mesenchymal tumors of the esophagus. Genetic analysis is crucial to establish the diagnosis and can be successfully performed on formalin-fixed, paraffin-embedded material and hematopoietic tissue.

Epstein-Barr Virus Gastritis: Rare or Rarely Sampled? A Case Report

Owens, S. R., Walls, A., Krasinskas, A., Rund, C. R. — Wed, 21 May 2008 22:19:42 PDT

Gastritis related to Epstein-Barr virus infection has been rarely reported in the literature. However, one population, arguably, most likely to be infected with the virus (young patients) is unlikely to undergo major diagnostic procedures that would yield pathological specimens containing the characteristic findings. Thus, the disease may be rarely sampled rather than truly rare. Furthermore, any patient with Epstein-Barr virus–related gastritis may manifest clinical and histological findings that can mimic hematolymphoid neoplasia. Therefore, care must be taken and a high level of clinical suspicion employed to avoid a misdiagnosis of lymphoma in these cases. Such a case is reported, with clinicopathological findings and follow-up information indicative of Epstein-Barr virus as the etiology of a clinically and histopathologically significant gastritis containing large, atypical immunoblast-like cells.