Desmoplastic Small Round Cell Tumor

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Retrieved

2022-04-26

Source

Gard

Trials

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Genes

WT1, EWSR1, FLI1, DES, IGF1R, TWIST1, SS18, PAX7, PAX3, CIC, BCOR, CCNB3, IL2, SYT1, FLII, FOXO1, KTWS, ERG, MUC16, SLC29A4, SSX2B, PATZ1, NES, TNFRSF10A, TNFSF10, TEC, CD6, PDGFA, TSPAN7, SSX2

WT1, EWSR1, FLI1, DES, IGF1R, TWIST1, SS18, PAX7, PAX3, CIC, BCOR, CCNB3, IL2, SYT1, FLII, FOXO1, KTWS, ERG, MUC16, SLC29A4, SSX2B, PATZ1, NES, TNFRSF10A, TNFSF10, TEC, CD6, PDGFA, TSPAN7, SSX2, SSX1, SMARCB1, CCN2, NKX2-2, NFATC2, CD99, IL15, GRN, GNA12, FUS, ETV4, ETV1, DUX4

Drugs

(-)-trans-3-(5,6-dihydro-4H-pyrrolo[3,2,1-ij]quinolin-1yl)-4-(1H-indol-3-yl) pyrrolidine-2, 5-dione, (1-methyl-2-nitro-1H-imidazole-5-yl)methyl N,N'-bis(2-bromoethyl) diamidophosphate, 16-base single-stranded peptide nucleic acid oligonucleotide linked to a 7-amino acid peptide, 18-(p-(, 4-oxo-4H-chromene-2-carboxylic acid (2-(2-4-(2-(6,7-dimethoxy-3,4-dihydro-1H-isoquinolin-2-yl)-ethyl)-phenyl-2H-tetrazol-5-yl)-4,5-dimethoxy-phenyl)-amide, 7-ethyl-10-hydroxy-camptothecin, Anlotinib, Autologous CD4+ and CD8+ T cells transduced with a lentiviral vector encoding an affinity enhanced T cell receptor specific to MAGE-A4, Autologous CD4+ and CD8+ T cells transduced with lentiviral vector containing an affinity-enhanced T-cell receptor targeting the New York esophageal antigen-1, Brostallicin, Camsirubicin, Crenolanib besylate, Deforolimus, Doxorubicin hydrochloride (liposomal) ( DOXIL ), Doxorubicin(6-maleimidocaproyl)hydrazone, Fenretinide, Fibromun, Genetically modified serotype 5/3 adenovirus coding for granulocyte-macrophage colony-stimulating factor, Human/murine chimeric monoclonal antibody against endoglin, Larotrectinib ( VITRAKVI ), Mammalian target of rapamcyin (mTOR) inhibitor, N-acetylsarcosyl-glycyl-L-valyl-D-allo-isoleucyl-L-threonyl-L-norvalyl-L-isoleucyl-L-arginyl-L-prolyl-N-ethylamide, Olaratumab ( LARTRUVO ), Ombrabulin, Paclitaxel ( TAXOL ), Palifosfamide, Propranolol hydrochloride ( HEMANGEOL ), Sindbis virus envelope pseudotyped lentiviral vector encoding New York oesophageal squamous cell carcinoma-1 protein, Trabectedin ( YONDELIS ), Vinorelbine tartrate, Yttrium (90Y)-DTPA-radiolabelled chimeric monoclonal antibody against frizzled homologue 10

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Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and 22, which is important in differentiating from other similar tumors. The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited.

Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation. Treatment options may include: chemotherapy, radiation therapy, surgery, and stem cell transplantation.