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

被引：7

作者：

Cobanoglu, H. Bengu ^{[1
]}

Hanna, Ehab Y. ^{[2
]}

Bell, Diana ^{[3
]}

Esmaeli, Bita ^{[4
]}

机构：

[1] Karadeniz Tech Univ, Sch Med, Dept Otorhinolaryngol Head & Neck Surg, Trabzon, Turkey

[2] Univ Texas MD Anderson Canc Ctr, Dept Head & Neck Surg, Houston, TX 77030 USA

[3] Univ Texas MD Anderson Canc Ctr, Dept Pathol, Houston, TX 77030 USA

[4] Univ Texas MD Anderson Canc Ctr, Dept Plast Surg, Orbital Oncol & Ophthalm Plast Surg, Houston, TX 77030 USA

来源：

CURRENT ONCOLOGY REPORTS | 2017年 / 19卷 / 12期

关键词：

Desmoplastic; Chemotherapy; Surgery; Proton therapy; SURVIVAL; RADIOTHERAPY; FUSION;

D O I：

10.1007/s11912-017-0638-x

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

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.

引用

页数：6

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