Sclerosing spindle cell rhabdomyosarcoma in an adult: report of a new case and review of the literature.

Abstract:

: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.

journal_name

Int J Surg Pathol

authors

Gavino AC,Spears MD,Peng Y

doi

10.1177/1066896908327166

subject

Has Abstract

pub_date

2010-10-01 00:00:00

pages

394-7

issue

5

eissn

1066-8969

issn

1940-2465

pii

1066896908327166

journal_volume

18

pub_type

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