Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in young patients. To contribute to the understanding of these tumors, we reviewed the records of all patients treated for PNSTs at one institution over a 30-year period. METHODS: We reviewed the records of eight patients treated for benign PNSTs and 28 patients treated for 29 MPNSTs. We focused on the latter group, statistically testing several clinical factors for their significance in affecting survival. RESULTS: Five-year survival in patients with MPNSTs was 39%. The most significant prognostic factor was gross tumor resectability (p = 0.0004). Five-year survival for patients with resectable tumors was 65%, whereas no patient with unresectable disease survived > 25 months. Tumor grade, site, and patient race were also significant factors by univariate analysis but were not significant when adjusted for resectability. CONCLUSION: Gross tumor resection is crucial in treating malignant PNSTs. Supplemental radiation therapy is recommended for positive microscopic margins. More effective treatment is still being sought for unresectable disease.