Translocation (4;19)(q35;q13.1)–Associated Primitive round Cell Sarcoma: Report of a Case and Review of the Literature

Author:

Rakheja Dinesh1,Goldman Stanton2,Wilson Kathleen S.3,Lenarsky Carl2,Weinthal Joel2,Schultz Roger A.3

Affiliation:

1. Department of Pathology, Children's Medical Center, and UT Southwestern Medical Center, Dallas, TX, USA

2. Texas Oncology Pediatrics, Dallas, TX, USA

3. Department of Pathology, UT Southwestern Medical Center, Dallas, TX, USA

Abstract

We report the 4th case of a primitive round cell sarcoma with the translocation (4;19)(q35;q13.1) as the primary cytogenetic abnormality. This undifferentiated sarcoma shows some features of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), including a diffuse reactivity for FLI1, but it shows only focal and weak reactivity for CD99 and is negative for a rearrangement of EWS, the molecular signature of ES/PNET. Recognition of the histopathologic and cytogenetic features of this entity is necessary to avoid its misdiagnosis as ES/PNET, especially in small biopsy samples.

Publisher

SAGE Publications

Subject

General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology and Child Health

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