title: Trabectedin in advanced desmoplastic round cell tumors: a retrospective single-center series.
abstract: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy that occurs with unpredictable chemosensitivity and limited treatment options in the advanced setting. Prognosis is poor, and exploring new treatment options for such diseases is difficult because of its rarity. Clinical activity of trabectedin for advanced DSRCT was scarcely reported in the literature. Here, we report a series of six patients treated with trabectedin for an unresectable DSRCT. After receiving trabectedin, two patients had stable disease with a time to progression of 3 and 3.5 months; four patients experienced disease progression after one cycle, two of them could receive one and two patients another line regiment. Four patients experienced grade 3-4 adverse events, two grade 3 thrombocytopenia, and one neutropenic fever. Prognosis was poor with a median overall survival of 4 (range: 2-14) months. In our experience, trabectedin had limited activity in advanced DSRCT. Further studies are warranted to find effective treatments.
authors: Sarcoma Group, Gustave Roussy Cancer Campus, Villejuif, France.
authors: Benjamin
authors: B
authors: Verret
authors: Charles
authors: C
authors: Honore
authors: Sarah
authors: S
authors: Dumont
authors: Philippe
authors: P
authors: Terrier
authors: Julien
authors: J
authors: Adam
authors: Andrea
authors: A
authors: Cavalcanti
authors: Isabelle
authors: I
authors: Sourrouille
authors: Guillaume
authors: G
authors: Klausner
authors: Marion
authors: M
authors: Ahlenc-Gelas
authors: Nicolas
authors: N
authors: Kiavue
authors: Gabriella
authors: G
authors: Domitrescu
authors: Lamiae
authors: L
authors: El Amarti
authors: Olivier
authors: O
authors: Mir
authors: Axel
authors: A
authors: Le Cesne
mesh_terms: D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000627:therapeutic use*; D058405:Desmoplastic Small Round Cell Tumor / Q000188:drug therapy*; D004149:Dioxoles / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D008113:Liver Neoplasms / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D010534:Peritoneal Neoplasms / Q000188:drug therapy; D010997:Pleural Neoplasms / Q000188:drug therapy; D012189:Retrospective Studies; D044005:Tetrahydroisoquinolines / Q000627:therapeutic use*; D000077606:Trabectedin; D055815:Young Adult
chemical_list: D018906:Antineoplastic Agents, Alkylating; D004149:Dioxoles; D044005:Tetrahydroisoquinolines; D000077606:Trabectedin