Translocation (4;19)(q35;q13.1)-associated primitive round cell sarcoma: Report of a case and review of the literature

Dinesh Rakheja; Stanton Goldman; Kathleen S Wilson; Carl Lenarsky; Joel Weinthal; Roger A. Schuttz

doi:10.2350/07-06-0296.1

Translocation (4;19)(q35;q13.1)-associated primitive round cell sarcoma: Report of a case and review of the literature

Dinesh Rakheja, Stanton Goldman, Kathleen S Wilson, Carl Lenarsky, Joel Weinthal, Roger A. Schuttz

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32 Scopus citations

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.

Original language	English (US)
Pages (from-to)	239-244
Number of pages	6
Journal	Pediatric and Developmental Pathology
Volume	11
Issue number	3
DOIs	https://doi.org/10.2350/07-06-0296.1
State	Published - May 2008

Keywords

Cytogenetics
Ewing sarcoma
Primitive round cell sarcoma

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Pathology and Forensic Medicine

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10.2350/07-06-0296.1

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AU - Lenarsky, Carl

AU - Weinthal, Joel

AU - Schuttz, Roger A.

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AB - 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.

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Translocation (4;19)(q35;q13.1)-associated primitive round cell sarcoma: Report of a case and review of the literature

Abstract

Keywords

ASJC Scopus subject areas

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