Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations
Joint Authors
Bulbul, Ajaz
Fahy, Bridget Noel
Xiu, Joanne
Rashad, Sadaf
Mustafa, Asrar
Husain, Hatim
Hayes-Jordan, Andrea
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-11-01
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
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.
American Psychological Association (APA)
Bulbul, Ajaz& Fahy, Bridget Noel& Xiu, Joanne& Rashad, Sadaf& Mustafa, Asrar& Husain, Hatim…[et al.]. 2017. Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations. Complexity،Vol. 2017, no. 2017, pp.1-12.
https://search.emarefa.net/detail/BIM-1197706
Modern Language Association (MLA)
Bulbul, Ajaz…[et al.]. Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations. Complexity No. 2017 (2017), pp.1-12.
https://search.emarefa.net/detail/BIM-1197706
American Medical Association (AMA)
Bulbul, Ajaz& Fahy, Bridget Noel& Xiu, Joanne& Rashad, Sadaf& Mustafa, Asrar& Husain, Hatim…[et al.]. Desmoplastic Small Round Blue Cell Tumor: A Review of Treatment and Potential Therapeutic Genomic Alterations. Complexity. 2017. Vol. 2017, no. 2017, pp.1-12.
https://search.emarefa.net/detail/BIM-1197706
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1197706