desmoplastic small round cell tumor (Cancer)
Information
- Disease name
- desmoplastic small round cell tumor
- Disease ID
- DOID:6785
- Description
- "A small cell sarcoma that is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass." [url:https\://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor, url:https\://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/desmoplastic-small-round-cell-tumors]
Disease area statistics
[No Data.]
Chromosome band
[No Data.]
Annotation
| Genes | Mutation | Description | Source | Links |
|---|
| NCT ID | Status | Phase | Summary | Start date | Completion date |
|---|---|---|---|---|---|
| NCT04483778 | Active, not recruiting | Phase 1 | B7H3 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults | July 13, 2020 | December 2040 |
| NCT03600649 | Active, not recruiting | Phase 1 | Clinical Trial of SP-2577 (Seclidemstat) in Patients With Relapsed or Refractory Ewing or Ewing-related Sarcomas | June 4, 2018 | December 2025 |
| NCT01946529 | Active, not recruiting | Phase 2 | Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors | December 27, 2013 | July 2026 |
| NCT04145349 | Active, not recruiting | Phase 1/Phase 2 | CAMPFIRE: A Study of Ramucirumab (LY3009806) in Children and Young Adults With Desmoplastic Small Round Cell Tumor | January 22, 2020 | October 31, 2024 |
| NCT04095221 | Active, not recruiting | Phase 1/Phase 2 | A Study of the Drugs Prexasertib, Irinotecan, and Temozolomide in People With Desmoplastic Small Round Cell Tumor and Rhabdomyosarcoma | September 17, 2019 | September 2024 |
| NCT00436657 | Completed | Phase 1 | Continuous Hyperthermic Peritoneal Perfusion (CHPP) With Cisplatin for Children With Peritoneal Cancer | February 2007 | April 2011 |
| NCT02982941 | Completed | Phase 1 | Enoblituzumab (MGA271) in Children With B7-H3-expressing Solid Tumors | December 2016 | May 22, 2019 |
| NCT05266196 | Enrolling by invitation | Phase 1/Phase 2 | A Rollover Protocol to Allow for Continued Access to the LSD1 Inhibitor Seclidemstat (SP-2577) | January 15, 2022 | December 2025 |
| NCT06456359 | Not yet recruiting | Phase 2 | Pasireotide as Maintenance Treatment in Synovial Sarcoma and Desmoplastic Small Round Cell Tumor | September 2024 | October 2028 |
| NCT06277154 | Not yet recruiting | Phase 2 | MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma | February 2024 | February 2027 |
| NCT04530487 | Recruiting | Phase 2 | Donor Stem Cell Transplant After Chemotherapy for the Treatment of Recurrent or Refractory High-Risk Solid Tumors in Pediatric and Adolescent-Young Adults | August 19, 2020 | May 9, 2025 |
| NCT03618381 | Recruiting | Phase 1 | EGFR806 CAR T Cell Immunotherapy for Recurrent/Refractory Solid Tumors in Children and Young Adults | June 18, 2019 | June 2040 |
| NCT04897321 | Recruiting | Phase 1 | B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR) | July 6, 2022 | March 1, 2027 |
| NCT04690374 | Recruiting | Registry to Collect Health Information About Desmoplastic Small Round Cell Tumor | December 22, 2020 | December 2027 | |
| NCT03967834 | Recruiting | N/A | Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group | April 26, 2021 | April 2031 |
| NCT04022213 | Recruiting | Phase 2 | A Study of the Drug I131-Omburtamab in People With Desmoplastic Small Round Cell Tumors and Other Solid Tumors in the Peritoneum | July 15, 2019 | July 2024 |
| NCT05918640 | Recruiting | Phase 1/Phase 2 | Lurbinectedin in FET-Fused Tumors | July 27, 2023 | July 30, 2028 |
| NCT01125449 | Suspended | Phase 2 | Study of High Dose Intravenous (IV) Ascorbic Acid in Measurable Solid Tumor Disease | January 2011 | December 2014 |
| NCT04901806 | Terminated | Phase 1 | Study of PBI-200 in Subjects With NTRK-Fusion-Positive Solid Tumors | July 20, 2021 | July 26, 2023 |
| NCT02982486 | Unknown status | Phase 2 | A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma | December 2017 | December 2020 |
| NCT02834169 | Unknown status | French National Registry of Rare Peritoneal Surface Malignancies | October 2010 | December 2018 |
- Disase is a (Disease Ontology)
- DOID:3098
- Cross Reference ID (Disease Ontology)
- GARD:6265
- Cross Reference ID (Disease Ontology)
- ICDO:8806/3
- Cross Reference ID (Disease Ontology)
- NCI:C8300
- Exact Synonym (Disease Ontology)
- desmoplastic small round-cell neoplasm
- OrphaNumber from OrphaNet (Orphanet)
- 83469
- MeSH unique ID (MeSH (Medical Subject Headings))
- D058405