Key result
Vinorelbine, cyclophosphamide, and temsirolimus yield 100% partial response in relapsed desmoplastic small round cell tumors.
Why the study?
Patients with relapsed desmoplastic small round cell tumor have a poor prognosis and no standard therapies are established.
Does a regimen of vinorelbine, cyclophosphamide, and temsirolimus improve outcomes in patients with relapsed desmoplastic small round cell tumor?
Case Report (n=5)
Does a regimen of vinorelbine, cyclophosphamide, and temsirolimus improve outcomes in patients with relapsed desmoplastic small round cell tumor?
Answers are grounded in this paper and cite their sources.
A combination of vinorelbine, cyclophosphamide, and temsirolimus showed preliminary efficacy in a small series of patients with relapsed desmoplastic small round cell tumor.
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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Tarek et al. (2017) conducted a case report in Relapsed desmoplastic small round cell tumor (DSRCT) (n=5). Vinorelbine, cyclophosphamide, and temsirolimus (VCT) was evaluated on Partial response. Vinorelbine, cyclophosphamide, and temsirolimus yielded a partial response in all 5 patients with relapsed desmoplastic small round cell tumor (median time to progression 8.5 months).
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