Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen

Nidale Tarek, Andrea Hayes-Jordan, Laura Salvador, Mary F. McAleer, Cynthia E. Herzog, Winston W. Huh

Research output: Contribution to journalArticlepeer-review

12 Scopus citations

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal tumor that typically presents with multiple abdominal masses. Initial treatment is multimodal in nature. Patients with relapsed DSRCT have a poor prognosis, and there are no standard therapies. We report our experience with five patients treated with vinorelbine, cyclophosphamide, and temsirolimus (VCT). Median number of VCT courses delivered was 7 (range 4–14 courses), and partial response was observed in all patients. Median time to progression or relapse was 8.5 months (range 7–16 months). Neutropenia and mucositis were most common toxicities (n = 4 each).

Original languageEnglish (US)
Article numbere26768
JournalPediatric Blood and Cancer
Volume65
Issue number1
DOIs
StatePublished - Jan 2018

Keywords

  • childhood cancer
  • desmoplastic
  • recurrence
  • sarcoma
  • temsirolimus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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