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lüll Malignant peripheral nerve sheath tumours in neurofibromatosis 1 Evans DG; Baser ME; McGaughran J; Sharif S; Howard E; Moran AJ Med Genet 2002[May]; 39 (5): 311-4BACKGROUND: Cross sectional studies have shown that 1-2% of patients with neurofibromatosis 1 (NF1) develop malignant peripheral nerve sheath tumours (MPNST). However, no population based longitudinal studies have assessed lifetime risk. METHODS: NF1 patients with MPNST were ascertained from two sources for our north west England population of 4.1 million in the 13 year period 1984-1996: the North West Regional NF1 Register and review of notes of patients with MPNST in the North West Regional Cancer Registry. RESULTS: Twenty-one NF1 patients developed MPNST, equivalent to an annual incidence of 1.6 per 1000 and a lifetime risk of 8-13%. There were 37 patients with sporadic MPNST. The median age at diagnosis of MPNST in NF1 patients was 26 years, compared to 62 years in patients with sporadic MPNST (p<0.001). In Kaplan-Meier analyses, the five year survival from diagnosis was 21% for NF1 patients with MPNST, compared to 42% for sporadic cases of MPNST (p=0.09). One NF1 patient developed two separate MPNST in the radiation field of a previous optic glioma. CONCLUSION: The lifetime risk of MPNST in NF1 is much higher than previously estimated and warrants careful surveillance and a low threshold for investigation.|Adolescent[MESH]|Adult[MESH]|Aged[MESH]|England/epidemiology[MESH]|Female[MESH]|Humans[MESH]|Incidence[MESH]|Longitudinal Studies[MESH]|Male[MESH]|Middle Aged[MESH]|Neurofibromatosis 1/diagnosis/*epidemiology/mortality[MESH]|Risk Assessment[MESH]|Survival Rate[MESH] |