This Article Full Text (PDF) All Versions of this Article: 1066896908319444v1 17/2/135    most recent References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Houreih, M. A. Articles by Banerjee, S. S. Search for Related Content PubMed PubMed Citation Articles by Houreih, M. A. Articles by Banerjee, S. S. Pubmed/NCBI databases Medline Plus Health Information Lymphoma Social Bookmarking                         What's this?

Alveolar Rhabdomyosarcoma With Neuroendocrine/Neuronal Differentiation: Report of 3 Cases

Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom

Amy Y. Lin, MD

Department of Pathology, University of Illinois at Chicago, Chicago, Illinois

Brian Eyden, PhD

Department of Pathology, University of Illinois at Chicago, Chicago, Illinois, brian.eyden{at}christie.nhs.uk

Lia P. Menasce, PhD, FRCPath

Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom

James Harrison, MBChB, FRCPath

Department of Pathology, Royal Albert Edward Infirmary, Wigan, United Kingdom

David Jones, PhD

Department of Diagnostic Molecular Pathology, Royal Manchester Children's Hospital NHS Trust, Pendlebury, United Kingdom

Robert Folberg, MD

Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom

Gregorio Chejfec, MD

Department of Pathology, University of Illinois at Chicago, Chicago, Illinois

S. Sankar Banerjee, MD, FRCPath

Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom

The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation. Specimens of 3 cases of alveolar rhabdomyosarcoma were studied using histologic, immunohistochemical, ultrastructural, and molecular genetic techniques. The patients were a 19-year-old man with metastatic alveolar rhabdomyosarcoma in a groin lymph node, a 16-year-old girl with alveolar rhabdomyosarcoma of the perineum, and a 20-year-old man with recurrent orbital alveolar rhabdomyosarcoma. Microscopically, case 1 was composed of compact sheets of medium to large tumor cells. Cases 2 and 3 were small blue round cell tumors. Cases 1 and 3 were solid throughout, whereas case 2 demonstrated alveolar and solid architecture. By immunohistochemistry, the following markers were positive: desmin (3/3), myogenin (3/3), synaptophysin (3/3), and chromogranin (2/3). Ultrastructurally, sarcomeric filaments were seen in all cases, while neuroendocrine granules were detected only in case 1. PAX:FKHR fusion transcript was identified in case 2, case 3 had a variant PAX3 transcript, and case 1 was negative. The data presented expands the known differentiation of alveolar rhabdomyosarcoma.

Key Words: alveolar rhabdomyosarcoma • neuroendocrine/neuronal differentiation • immunohistochemistry • electron microscopy • molecular genetics

This version was published on April 1, 2009

International Journal of Surgical Pathology, Vol. 17, No. 2, 135-141 (2009)
DOI: 10.1177/1066896908319444


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?