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Pooling twelve EORTC trials showed that malignant peripheral nerve sheath tumours respond to doxorubicin-ifosfamide about as well as other sarcomas, though survival is shorter, and that NF1-associated tumours may respond less.
Retrospective analysis of 175 patients with malignant peripheral nerve sheath tumour among 2,675 soft tissue sarcoma patients treated with first-line anthracycline-based chemotherapy in EORTC trials.
Response rate was 21 percent in MPNST compared with 22 percent in other sarcomas, with the highest response to doxorubicin-ifosfamide; median overall survival was 48 weeks against 51 weeks, and NF1-associated tumours tended to respond less.
Doxorubicin-ifosfamide is the standard first-line chemotherapy for advanced MPNST, and the analysis supports treating it like other high-grade sarcomas while recognising its shorter survival.