Malignant peripheral nerve sheath tumors: a report from children’s oncology group study ARST0332

Crane JN, Xue W, Qumseya A, Barkauskas DA, Chau K, Tan SY, Hiniker S, Dasgupta R, Venkatramani R, Spunt SL, Weiss AR, Laetsch TW. Malignant peripheral nerve sheath tumors: a report from children’s oncology group study ARST0332. J Natl Cancer Inst. 2025 Dec 12:djaf359. doi: 10.1093/jnci/djaf359. Epub ahead of print. PMID: 41386267; PMCID: PMC12993871.

The cornerstone of the treatment of malignant peripheral nerve sheath tumors is surgical resection. Radiation and chemotherapy are variably employed. The optimal treatment remains uncertain, particularly for unresectable or metastatic disease and patients with neurofibromatosis type 1 (NF-1). We present data for 58 patients with newly diagnosed malignant peripheral nerve sheath tumors enrolled on the Children’s Oncology Group study ARST0332. Patients were treated with risk-adapted therapy including surgery with or without radiotherapy and ifosfamide and doxorubicin chemotherapy.