Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen |
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| Authors: | Nidale Tarek Andrea Hayes‐Jordan Laura Salvador Mary F. McAleer Cynthia E. Herzog Winston W. Huh |
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| Affiliation: | 1. Department of Pediatrics and Adolescent Medicine, Children's Cancer Institute, American University of Beirut Medical Center, Beirut, Lebanon;2. Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas;3. Division of Pediatrics, University of Texas MD Anderson Cancer Center, Houston, Texas;4. Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas |
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| 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). |
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| Keywords: | childhood cancer desmoplastic recurrence sarcoma temsirolimus |
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