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. © 2013 Springer-Verlag France.