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ID | DOID:6785 |
Name | desmoplastic small round cell tumor |
Definition | 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. |
Source | DiseaseOntology.org |
Alt Ids | |
Path | disease disease of cellular proliferation cancer cell type cancer sarcoma small cell sarcoma desmoplastic small round cell tumor |
Molecular Profile | Therapy | Indication/Tumor Type | Response Type | Profile Response Detail |
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Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
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NCT04741438 | Phase III | Ipilimumab + Nivolumab Pazopanib | Efficacy of the Combination of Nivolumab and Ipilimumab as a Treatment in Patients With Sarcoma of Rare Subtype (RAR-Immune) | Recruiting | FRA | 0 |
NCT04901806 | Phase Ib/II | PBI-200 | Study of PBI-200 in Subjects With NTRK-Fusion-Positive Solid Tumors | Terminated | USA | ITA | GBR | FRA | ESP | DNK | DEU | AUS | 3 |
NCT05004116 | Phase Ib/II | Irinotecan + Repotrectinib + Temozolomide | A Study of Repotrectinib in Combination With Chemotherapy in Children and Young Adults With Solid Tumor Cancer | Recruiting | USA | 0 |
NCT05131386 | Phase II | Dexamethasone + Trabectedin | Multicohort Trial of Trabectedin and Low-dose Radiation Therapy in Advanced/Metastatic Sarcomas (SYNERGIAS) | Recruiting | ESP | 0 |
NCT05253131 | Phase Ib/II | AZD5153 + Durvalumab + Selumetinib AZD5153 + Selumetinib | Trial of Selumetinib and AZD5153 With Durvalumab for Sarcomas | Not yet recruiting | USA | 0 |
NCT05275426 | Phase II | Gemcitabine + LY2880070 | A Study of LY2880070 and Gemcitabine in People With Ewing Sarcoma,Ewing-Like Sarcoma, and Desmoplastic Small Round Cell Tumor | Recruiting | USA | 0 |
NCT05468359 | Phase Ib/II | Atezolizumab + Bevacizumab + Cyclophosphamide + Sorafenib | Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients | Recruiting | USA | 0 |
NCT06456359 | Phase II | Pasireotide | Pasireotide as Maintenance Treatment in Synovial Sarcoma and Desmoplastic Small Round Cell Tumor (PAMSARC) | Not yet recruiting | DEU | 0 |