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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

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Molecular Profile Therapy Indication/Tumor Type Response Type Profile Response Detail
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
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