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01-12-2017 | Head and Neck Cancers (EY Hanna, Section Editor)

Desmoplastic Small Round Cell Tumor Presenting as an Ocular Mass: Unusual Localization and Remarkable Surgical Approach

Authors: H. Bengu Cobanoglu, Ehab Y. Hanna, Diana Bell, Bita Esmaeli

Published in: Current Oncology Reports | Issue 12/2017

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare tumor that was first described by Gerald and Rosai in 1989 as a mesenchymal entity. This tumor has a unique translocation t (11:22) (p:13, q:12) resulting in EWS/WT1 gene fusion that is diagnostic for DSCRT. The overall prognosis for desmoplastic small round cell tumor remains extremely poor, with reported rates of death as high as 90%. Although the majority of patients undergo chemotherapy following surgery, the prognosis has been shown to be independent of whether the surgical process preceded or followed chemotherapy. In this review, we provide insights for the management of DSCRT that requires aggressive multimodality therapy.

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Title: Desmoplastic Small Round Cell Tumor Presenting as an Ocular Mass: Unusual Localization and Remarkable Surgical Approach
Authors: H. Bengu Cobanoglu
Ehab Y. Hanna
Diana Bell
Bita Esmaeli
Publication date: 01-12-2017
Publisher: Springer US
Published in: Current Oncology Reports / Issue 12/2017
Print ISSN: 1523-3790
Electronic ISSN: 1534-6269
DOI: https://doi.org/10.1007/s11912-017-0638-x

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