Alveolar Soft Tissue Sarcoma

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Retrieved

2021-01-23

Source

Orphanet

Trials

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Genes

ASPSCR1, TFE3, MET, CD38, CD68, MLH1, MSH2, CTSK, CTNNB1, CSNK1D, ACTB, MYOD1, VEGFA, TP53, PAX8, TIMP2, SPG7, SET, VIM, XRCC1, CD163, TFEB, PROM1, PER2, PSMC2, NR1I3, TRIM13, PDPN, NES, ANGPTL2

ASPSCR1, TFE3, MET, CD38, CD68, MLH1, MSH2, CTSK, CTNNB1, CSNK1D, ACTB, MYOD1, VEGFA, TP53, PAX8, TIMP2, SPG7, SET, VIM, XRCC1, CD163, TFEB, PROM1, PER2, PSMC2, NR1I3, TRIM13, PDPN, NES, ANGPTL2, BHLHE41, COG8, TRIM63, TMED6, RET, PCSK1, PSMA4, ATN1, ANG, APC, ARR3, ASS1, BSG, CALD1, CASR, CD34, CXADR, DES, MLANA, PRKAR1A, HIF1A, HTC2, ITGB1, KDR, MDK, MYOG, TBC1D25, ALDH1A1, PDGFRB, ABCB1, CXADRP1

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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A rare soft tissue sarcoma characterized by a slowly growing, painless space-occupying lesion, composed of large, uniform, epitheloid cells arranged in solid nests and/or alveolar structures, separated by thin, sinusoidal vessels. The tumor mostly affects adolescents and young adults. Early metastasis, most commonly to the lung, bones, and brain, is a characteristic feature and relevant prognostic factor, together with age at presentation and tumor size, while histological features have no prognostic significance.