Targeted Oncology

, Volume 8, Issue 3, pp 211–213 | Cite as

Clinical activity of sunitinib in patients with advanced desmoplastic round cell tumor: a case series

  • Antoine ItalianoEmail author
  • Michèle Kind
  • Angela Cioffi
  • Robert G. Maki
  • Binh Bui
Day-to-Day Practice


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.


Desmoplastic round cell tumor Sunitinib Treatment 


Conflict of interest

The authors declare that they have no conflict of interest.


  1. 1.
    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, LyonGoogle Scholar
  2. 2.
    Hayes-Jordan A, Anderson PM (2011) The diagnosis and management of desmoplastic small round cell tumor: a review. Curr Opin Oncol 23:385–389PubMedCrossRefGoogle Scholar
  3. 3.
    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 10016CrossRefGoogle Scholar
  4. 4.
    Chow LQM, Eckhardt SG (2007) Sunitinib: from rational design to clinical efficacy. J Clin Oncol 25:884–896PubMedCrossRefGoogle Scholar
  5. 5.
    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 27:3154–3160PubMedCrossRefGoogle Scholar
  6. 6.
    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 29:78–85PubMedGoogle Scholar

Copyright information

© Springer-Verlag France 2013

Authors and Affiliations

  • Antoine Italiano
    • 1
    Email author
  • Michèle Kind
    • 2
  • Angela Cioffi
    • 3
  • Robert G. Maki
    • 4
    • 5
  • Binh Bui
    • 1
  1. 1.Department of Medical OncologyInstitut BergonieBordeauxFrance
  2. 2.Department of RadiologyInstitut BergoniéBordeauxFrance
  3. 3.Department of MedicineInstitut Gustave RoussyVillejuifFrance
  4. 4.Sarcoma ServiceMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  5. 5.Mount Sinai School of MedicineNew YorkUSA

Personalised recommendations