Desmoplastic Small Round Cell Tumor: RT-PCR Analysis and Immunohistochemical Detection of the Wilm's Tumor Gene WT1

doi:10.1016/S0344-0338(98)80128-3

Pathology - Research and Practice

Volume 194, Issue 10, 1998, Pages 693-700

https://doi.org/10.1016/S0344-0338(98)80128-3 Get rights and content

Summary

Desmoplastic small round cell tumor is an aggressive neoplasm first described in 1991. Recently, a reciprocal translocation t( 11 ;22)(p 13 ;q 12) has been characterized by conventional cytogenetic studies and molecular analysis. This translocation involves the Ewing’s sarco- ma gene on chromosome 22 and the Wilms’ tumor gene WT1 on chromosome 11. The chimeric transcript corre- sponding to the fusion gene could be detected by the re- verse transcriptase-polymerase chain reaction (RT- PCR). Using an anti-WTl antibody, the WT1 part of the putative chimeric protein could be recognized by im- munohistochemistry. We describe two well-character- ized cases of intraabdominal desmoplastic small round cell tumor in two male patients aged 14 and 28 with both RT-PCR analysis and immunostaining for WT1. In this report, we insist on the necessity to increase the RT- PCR analysis in DSRCT in order to obtain a precise dif- ferential diagnosis. In addition, WT1 immunostaining may serve as a useful diagnostic marker for DSRCT.

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Desmoplastic Small Round Cell Tumor: RT-PCR Analysis and Immunohistochemical Detection of the Wilm's Tumor Gene WT1

Summary

Hum Pathol

A light microscopic, immunocytochemical, ultrastructural, and flow cytometric study. Hum Pathol

Cancer Genet Cytogenet

Masse mésocolique chez un adolescent

Ann Pathol

Detection of the EWS/WT1 Gene fusion by Reverse Transcriptase-Polymerase Chain Reaction in the diagnosis of intra-abdominal desmoplastic small round cell tumor

Am J Surg Pathol

Molecular assays for chromosomal translocations in the diagnosis of pediatric soft tissue sarcomas

JAMA

Fusion of the EWS1 and WT1 genes as a result of the t( 11 ;22)(p 13 ;q 12) Translocation in desmoplastic small round cell tumors

Med Ped Oncol

Effusion cytology of desmoplastic small round cell tumor of the pleura

A case report. Acta Cytol

Modulation of DNA binding specificity by alternative splicing of the Wilms tumor wtl gene transcript

Science

Translocation (Il;22)(pl3;ql2): primary change in intra-abdominal desmoplastic small round cell tumor

Genes Chrom Cancer