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Malignant peripheral nerve sheath tumours associated with von Recklinghausen's neurofibromatosis
1University of Pittsburgh Medical Center, PA 15213, USA.
Aims:
A retrospective study was conducted of patients with von Recklinghausen's neurofibromatosis who developed malignant peripheral nerve sheath tumours to determine their clinical characteristics, treatment outcome and prognostic factors.
Methods:
During the period 1987-1995, 29 patients with von Recklinghausen's neurofibromatosis (VRN) were seen at the Royal Marsden Hospital, of whom 23 had malignant peripheral nerve sheath tumours (MPNSTs).
Results:
The median age of the patients with MPNSTs was 30 years and the sex distribution was equal. The commonest site was the lower extremity, with a tendency for tumours to occur in the proximal parts. A nerve of origin was identified in 59% of the tumours and heterologous differentiation was present in 26%. Multiple primary MPNSTs occurred in four patients (17%) and other malignant tumours developed in three (13%). The overall 5-year survival was 35% with a median survival of 36 months. The incidence of local recurrence was 38% and 75% of them occurred within 2 years.
Conclusions:
MPNSTs which develop in association with VRN occur in a younger age group, tend to be of a higher histological grade and are associated with a very poor prognosis. Tumour size > or = 10 cm (P=0.003) and high tumour grade (P=0.003) were adverse independent prognostic factors for overall survival.