Desmoplastic Small Round Cell Tumor

A rare, aggressive soft tissue sarcoma that primarily affects adolescents and young adults. It is most commonly found in the abdomen.
Also Known As:
Desmoplastic Small Cell Tumor; Desmoplastic Small Round-Cell Tumor; Desmoplastic Small-Cell Tumor; Desmoplastic Small-Cell Tumors; Small-Cell Tumor, Desmoplastic; Small-Cell Tumors, Desmoplastic; Tumor, Desmoplastic Small-Cell; Tumors, Desmoplastic Small-Cell
Networked: 93 relevant articles (2 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Rhabdomyosarcoma
2. Neoplasms (Cancer)
3. Ewing's Sarcoma (Sarcoma, Ewing)
4. Neuroblastoma
5. Primitive Neuroectodermal Tumors (PNET)


1. Hayes-Jordan, Andrea: 3 articles (08/2015 - 01/2008)
2. Haber, Daniel A: 3 articles (01/2008 - 12/2002)
3. Wolden, Suzanne L: 2 articles (01/2015 - 09/2002)
4. Modak, Shakeel: 2 articles (01/2015 - 12/2002)
5. Kushner, Brian H: 2 articles (01/2015 - 09/2002)
6. Petersson, Fredrik: 2 articles (01/2015 - 01/2011)
7. La Quaglia, Michael P: 2 articles (02/2014 - 09/2002)
8. Subbiah, Vivek: 2 articles (01/2013 - 04/2011)
9. Anderson, Pete M: 2 articles (01/2013 - 04/2011)
10. Ferrari, Andrea: 2 articles (09/2012 - 11/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Desmoplastic Small Round Cell Tumor:
1. Cyclophosphamide (Cytoxan)FDA LinkGeneric
2. vinorelbine (Navelbine)FDA LinkGeneric
3. RNA-Directed DNA Polymerase (Reverse Transcriptase)IBA
4. Nicotinic Receptors (Nicotinic Acetylcholine Receptor)IBA
5. 17-(allylamino)-17-demethoxygeldanamycin (17AAG)IBA
6. DesminIBA
7. Keratins (Keratin)IBA
8. ParaffinIBA
9. Transcription Factors (Transcription Factor)IBA
10. RNA (Ribonucleic Acid)IBA
05/01/2014 - "Total RNA from four ES cell lines plus 33 clinical samples representing ES, alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, and congenital fibrosarcoma tumors was subjected to ChildSeq-RNA. "
05/01/2014 - "We developed a next-generation sequencing-based assay designated ChildSeq-RNA that uses the Ion Torrent platform to screen for EWSR1-FLI1 and EWSR1-ERG, PAX3-FOXO1 and PAX7-FOXO1, EWSR1-WT1, and ETV6-NTRK3 fusions of Ewing sarcoma (ES), alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, and congenital fibrosarcoma, respectively. "
08/01/2002 - "RT-PCR was used to test tumor RNA for fusion transcripts characteristic of malignant round cell tumors (including Ewing sarcoma/primitive neuroectodermal tumor, desmoplastic small round cell tumor, and alveolar rhabdomyosarcoma), spindle cell tumors (including synovial sarcoma and congenital fibrosarcoma), and fatty tumors (myxoid liposarcoma). "
12/01/1995 - "One desmoplastic small round cell tumor was found to have a variant EWS-WT1 chimeric product that included exon 8 of EWS EWS-FLI-1 chimeric RNA was present in all Ewing's sarcoma/primitive neuroectodermal tumor and not identified in any other tumor types, including desmoplastic small round cell tumor. "
09/01/1995 - "Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). "

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Radiotherapy
3. Combined Modality Therapy
4. Laparotomy
5. Immunotherapy