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J Med Genet. 2002 May;39(5):311-4.

Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

Author information

  • 1University Department of Medical Genetics, St Mary's Hospital, Manchester M13 0JH, UK. Gevans@central.cmht.nwest.nhs.uk

Abstract

BACKGROUND:

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.

PMID:
12011145
[PubMed - indexed for MEDLINE]
PMCID:
PMC1735122
Free PMC Article
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