title: Temsirolimus for metastatic desmoplastic small round cell tumor.
abstract: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive neoplasm that mostly occurs in young males. No curative treatment options currently exist for this type of tumor and long-term survival remains poor. In vitro rapamycin induces apoptotic death of JN-DSRCT-1 cells, a possible model for desmoplastic small round cell tumors in which the EWS gene is fused to the WT1 gene. We therefore demonstrate the prolonged activity of temsirolimus, an mTOR-inhibitor, in a patient with DSRCT.
authors: Department of Medical Oncology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. A.Thijs@aig.umcn.nl
authors: Anna M J
authors: AM
authors: Thijs
authors: Winette T A
authors: WT
authors: van der Graaf
authors: Carla M L
authors: CM
authors: van Herpen
mesh_terms: D000008:Abdominal Neoplasms / Q000473:pathology*; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy / Q000556:secondary; D008175:Lung Neoplasms / Q000188:drug therapy / Q000556:secondary; D008207:Lymphatic Metastasis; D008297:Male; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D020123:Sirolimus / Q000031:analogs & derivatives* / Q000627:therapeutic use; D058570:TOR Serine-Threonine Kinases / Q000037:antagonists & inhibitors*; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D047428:Protein Kinase Inhibitors; C401859:temsirolimus; D058570:TOR Serine-Threonine Kinases; D020123:Sirolimus