Malignant peripheral nerve sheath tumors in childhood: 13 cases from a single center

We aimed to review clinical characteristics, treatment results, and outcome for pediatric patients with malignant peripheral nerve sheath tumors (MPNSTs).

Files for 13 children diagnosed with MPNSTs and treated at our hospital between 1988 and 2009 were reviewed for clinical characteristics, treatment results, and outcome.

The median patient age was 11 years (range, 0.16 to 18 y; female/male: 6/7). The most common symptoms were palpable mass (7 of 13) and pain (3 of 13). Four patients had neurofibromatosis type 1. Physical findings at diagnosis were palpable mass (10 of 13), scoliosis (2 of 13), paraplegia/cranial nerve palsy (2 of 13), and stigmas of neurofibromatosis type 1. The primary sites were head and neck (5 of 13), abdomen (3 of 13), chest wall (2 of 13), abdominal wall (2 of 13), and extremities (1 of 13). According to the Intergroup Rhabdomyosarcoma Study system, 5 cases were classified as Intergroup Rhabdomyosarcoma Study group I, 6 were classified as group III, and 2 were classified as group IV. Five-year overall and event-free survival rates were 34.6% (±13.8) and 18.8% (±11.9), respectively. Overall survival rates did not differ upon addition of chemotherapy and/or radiotherapy to the surgery. Five-year survival rates were 42.9% (±18.9) in patients with total resection and 37.5% (±28.6) in patients with partial resection (P=0.08). Overall survival rates did not differ according to stages (P=0.8).

MPNSTs are rare in childhood and have an unfavorable prognosis. Whereas chemotherapy and/or radiotherapy had no promising effect on overall survival rates, complete resection seems to be the most effective treatment for MPNSTs. Further multicenter studies on the role of chemotherapy and/or radiotherapy for MPNSTs are warranted.