Translocation (4;19)(q35;q13.1)-associated primitive round cell sarcoma: report of a case and review of the literature.
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.
Rakheja
Dinesh
D
Department of Pathology, Children's Medical Center, and UT Southwestern Medical Center, Dallas, TX, USA. dinesh.rakheja@utsouthwestern.edu
Goldman
Stanton
S
Wilson
Kathleen S
KS
Lenarsky
Carl
C
Weinthal
Joel
J
Schultz
Roger A
RA
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002869:Chromosome Aberrations; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics*; D002894:Chromosomes, Human, Pair 4 / Q000235:genetics*; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary; D008297:Male; D018228:Sarcoma, Small Cell / Q000188:drug therapy / Q000235:genetics* / Q000556:secondary; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D014178:Translocation, Genetic