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.

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References
Fletcher CDM, Unni KK, Mertens F (eds) (2002) World Health Organisation classification of tumours. Pathology and genetics of tumours of soft tissue and bone. IARC Press, Lyon
Hayes-Jordan A, Anderson PM (2011) The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 23:385–389
Magnan HD, Chou T, LaQuaglia MP, Gerald W, Ladanyi M, Merchant MS (2009) Elevated expression of VEGFR-2 and VEGFA in desmoplastic small round cell tumor (DSRCT) and activity of bevacizumab and irinotecan in a xenograft model of DSRCT. J Clin Oncol 27:15s, suppl; abstr 10016
Chow LQM, Eckhardt SG (2007) Sunitinib: from rational design to clinical efficacy. J Clin Oncol 25:884–896
George S, Merriam P, Maki RG, Van den Abbeele AD, Yap JT, Akhurst T, Harmon DC, Bhuchar G, O'Mara MM, D'Adamo DR, Morgan J, Schwartz GK, Wagner AJ, Butrynski JE, Demetri GD, Keohan ML (2009) Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol 27:3154–3160
Froberg K, Brown RE, Gaylord H, Manivel C (1999) Intra-abdominal desmoplastic small round cell tumor: immunohistochemical evidence for up-regulation of autocrine and paracrine growth factors. Ann Clin Lab Sci 29:78–85
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Italiano, A., Kind, M., Cioffi, A. et al. Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series. Targ Oncol 8, 211–213 (2013). https://doi.org/10.1007/s11523-012-0251-8
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DOI: https://doi.org/10.1007/s11523-012-0251-8