Pediatric Malignant Peripheral Nerve Sheath Tumor: The Italian and German Soft Tissue Sarcoma Cooperative Group

MPNST is an aggressive tumor for which complete surgical resection is the mainstay of successful treatment. Postoperative radiotherapy may have a role in improving local control in patients with minimal residual tumor. The reported responses to primary chemotherapy suggest that it may be effective in patients with tumor considered unresectable at diagnosis.