Recurrent desmoplastic small round cell tumor responding to an mTOR inhibitor containing regimen
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John Wiley and Sons Inc.
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). © 2017 Wiley Periodicals, Inc.
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Keywords
Childhood cancer, Desmoplastic, Recurrence, Sarcoma, Temsirolimus, Adolescent, Adult, Antineoplastic combined chemotherapy protocols, Child, Cyclophosphamide, Desmoplastic small round cell tumor, Female, Humans, Male, Mucositis, Neoplasm recurrence, local, Neutropenia, Sirolimus, Vinblastine, Carboplatin, Creatinine, Dacarbazine, Dactinomycin, Doxorubicin, Epirubicin, Etoposide, Fluorodeoxyglucose f 18, Gemcitabine, Ifosfamide, Irinotecan, Mammalian target of rapamycin inhibitor, Paclitaxel, Temozolomide, Vincristine, Vinorelbine tartrate, Antineoplastic agent, Rapamycin, Abdomen, Abdominal cancer, Article, Cancer combination chemotherapy, Clinical article, Clinical assessment, Creatinine blood level, Cytoreductive surgery, Drug megadose, Fatigue, Fever, Human, Kidney injury, Liver cancer, Liver metastasis, Maintenance chemotherapy, Mucosa inflammation, Multimodality cancer therapy, Observational study, Pelvis cancer, Positron emission tomography-computed tomography, Priority journal, Retrospective study, School child, Tumor growth, Tumor recurrence, Analogs and derivatives, Chemically induced, Clinical trial, Diagnostic imaging