Open AccessGenomic and Proteomic Alterations in Desmoplastic Small Round Blue-Cell Tumors
Author(s) -
Ajaz Bulbul,
John Paul Shen,
Joanne Xiu,
Pablo Tamayo,
Hatim Husain
Publication year - 2018
Publication title -
jco precision oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.405
H-Index - 22
ISSN - 2473-4284
DOI - 10.1200/po.17.00170
Subject(s) - desmoplastic small round cell tumor , biology , computational biology , cancer research , genetics , chemotherapy
Desmoplastic small round blue-cell tumors (DSRCTs) are sarcomas that contain the t(11;22) (p13;q12) translocation EWS-WT1 fusion protein. Because this is a rare tumor type, prospective clinical trials in DSRCT are challenging. Patients are treated in a manner similar to those with Ewing sarcoma; however, differences in prognosis and clinical presentation suggest fundamental differences in biology and potentially different therapeutic implications. This study aimed to characterize the molecular characteristics of DSRCT tumors to explore unique therapeutic options for this extremely rare and aggressive cancer type.