First-line chemotherapy for malignant peripheral nerve sheath tumour versus other soft tissue sarcomas (EORTC pooled analysis)
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.
Overview
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.
- Objective response 21 percent in MPNST vs 22 percent in other sarcomas.
- Median overall survival 48 weeks; lower response in NF1-associated MPNST.
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 poorer prognosis.
- Retrospective pooled analysis across trials spanning decades.