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Sarcoma
Volume 2017, Article ID 1278268, 12 pages
https://doi.org/10.1155/2017/1278268
Review Article

Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations

1Department of Hematology/Oncology, Kymera Independent Physicians, Carlsbad, NM, USA
2Division of Internal Medicine, Department of Hematology/Oncology, Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX, USA
3Department of Surgery, University of New Mexico, Albuquerque, NM, USA
4Caris Life Sciences, Phoenix, AZ, USA
5All Saints University School of Medicine, Roseau, Dominica
6Acharya Shri Chander College of Medical Sciences and Hospital, Jammu, India
7Moores Cancer Center, University of California, San Diego, La Jolla, CA, USA
8Department of Pediatric Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX, USA

Correspondence should be addressed to Ajaz Bulbul; moc.liamg@lublubzaja

Received 9 June 2017; Accepted 17 September 2017; Published 1 November 2017

Academic Editor: Chandrajit Premanand Raut

Copyright © 2017 Ajaz Bulbul et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Desmoplastic small round blue cell tumors (DSRCTs) originate from a cell with multilineage potential. A molecular hallmark of DSRCT is the EWS-WT1 reciprocal translocation. Ewing sarcoma and DSRCT are treated similarly due to similar oncogene activation pathways, and DSRCT has been represented in very limited numbers in sarcoma studies. Despite aggressive therapy, median survival ranges from 17 to 25 months, and 5-year survival rates remain around 15%, with higher survival reported among those undergoing removal of at least 90% of tumor in the absence of extraperitoneal metastasis. Almost 100% of these tumors contain t(11;22) (p13;q12) translocation, and it is likely that EWS-WT1 functions as a transcription factor possibly through WT1 targets. While there is no standard protocol for this aggressive disease, treatment usually includes the neoadjuvant HD P6 regimen (high-dose cyclophosphamide, doxorubicin, and vincristine (HD-CAV) alternating with ifosfamide and etoposide (IE) chemotherapy combined with aggressively attempted R0 resection). We aimed to review the molecular characteristics of DSRCTs to explore therapeutic opportunities for this extremely rare and aggressive cancer type.