title: Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series.
abstract: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy with poor outcome occurring in adolescents and young adults. Therapeutic options for patients with advanced disease are limited. Preclinical studies have shown that VEGFR-2 and VEGFA are overexpressed in DSRCT and that DSRCT xenografts were highly responsive to anti-VEGF agents such as bevacizumab. We report here the clinical activity of sunitinib in eight patients with DSCRT. Our data suggest that sunitinib may be associated with clinical benefit even in heavily pretreated patients.
authors.affiliation: Department of Medical Oncology, Institut Bergonie, 229 Cours de l'Argonne, 33076, Bordeaux, France. a.italiano@bordeaux.unicancer.fr.
authors.forename: Antoine
authors.initials: A
authors.lastname: Italiano
authors.affiliation: Department of Radiology, Institut Bergonié, Bordeaux, France.
authors.forename: Michèle
authors.initials: M
authors.lastname: Kind
authors.affiliation: Department of Medicine, Institut Gustave Roussy, Villejuif, France.
authors.forename: Angela
authors.initials: A
authors.lastname: Cioffi
authors.affiliation: Sarcoma Service, Memorial Sloan-Kettering Cancer Center, New York, USA.|Mount Sinai School of Medicine, New York, NY, USA.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Maki
authors.affiliation: Department of Medical Oncology, Institut Bergonie, 229 Cours de l'Argonne, 33076, Bordeaux, France.
authors.forename: Binh
authors.initials: B
authors.lastname: Bui
mesh_terms: D000293:Adolescent; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D058405:Desmoplastic Small Round Cell Tumor / Q000188:drug therapy* / Q000473:pathology; D004334:Drug Administration Schedule; D006801:Humans; D007211:Indoles / Q000008:administration & dosage*; D008297:Male; D008875:Middle Aged; D011758:Pyrroles / Q000008:administration & dosage*; D000077210:Sunitinib; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; D000077210:Sunitinib