Radiation-Induced Malignant Peripheral Nerve Sheath Tumors: A Systematic Review

OBJECTIVE: Radiation-induced malignant peripheral nerve sheath tumors (MPNSTs) are an uncommon late risk of irradiation. We conducted the largest systematic review to date of individual patient data for patients with these tumors. METHODS: We conducted a systematic search using the PubMed database, and compiled a systematic literature review. We used Kaplan-Meier analysis and a log-rank test to estimate survival. RESULTS: We analyzed 65 radiation-induced and 26 radiation-associated MPNSTs in patients with neurofibromatosis. The mean ages of onset for primary lesions of the 2 types were 31.7 +/- 18.2 and 17.1 +/- 12.4 years, respectively (P = 0.0008). The latency periods between radiotherapy and onset of the 2 types of MPNSTs were 13.5 +/- 7.8 and 11.8 +/- 7.5 years, respectively (P = 0.3101). The median overall survival and 5-year survival were 11 months (6.8%) and 23 months (5.8%), respectively (P = 0.2168). Negative surgical margin and patient sex were variables retained for the prognosis of radiation-induced and radiationassociated MPNSTs. CONCLUSIONS: The prognosis of radiation-induced and radiation-associated MPNST was worse than that reported for de novo MPNSTs. Surgical complete resection is the mainstay for treatment of radiation-induced and radiationassociated MPNSTs. The risk of incidence of secondary MPNSTs in patients treated with radiotherapy warrants longer follow-up periods.