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.
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:NF1patients 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 NF1patients 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 NF1patients 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 NF1patients with MPNST, compared to 42% for sporadic cases of MPNST (p=0.09). One NF1patient 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.
Authors: R E Ferner; J D Lucas; M J O'Doherty; R A Hughes; M A Smith; B F Cronin; J Bingham Journal: J Neurol Neurosurg Psychiatry Date: 2000-03 Impact factor: 10.154
Authors: Trent R Hummel; Walter J Jessen; Shyra J Miller; Lan Kluwe; Victor F Mautner; Margaret R Wallace; Conxi Lázaro; Grier P Page; Paul F Worley; Bruce J Aronow; Elizabeth K Schorry; Nancy Ratner Journal: Clin Cancer Res Date: 2010-08-25 Impact factor: 12.531
Authors: Martha S Linet; Thomas L Slovis; Donald L Miller; Ruth Kleinerman; Choonsik Lee; Preetha Rajaraman; Amy Berrington de Gonzalez Journal: CA Cancer J Clin Date: 2012-02-03 Impact factor: 508.702
Authors: Stephanie J Byer; Jenell M Eckert; Nicole M Brossier; Buffie J Clodfelder-Miller; Amy N Turk; Andrew J Carroll; John C Kappes; Kurt R Zinn; Jeevan K Prasain; Steven L Carroll Journal: Neuro Oncol Date: 2010-11-12 Impact factor: 12.300
Authors: Syed J Kazmi; Stephanie J Byer; Jenell M Eckert; Amy N Turk; Richard P H Huijbregts; Nicole M Brossier; William E Grizzle; Fady M Mikhail; Kevin A Roth; Steven L Carroll Journal: Am J Pathol Date: 2013-01-13 Impact factor: 4.307
Authors: Carlos E Prada; Edwin Jousma; Tilat A Rizvi; Jianqiang Wu; R Scott Dunn; Debra A Mayes; Jose A Cancelas; Eva Dombi; Mi-Ok Kim; Brian L West; Gideon Bollag; Nancy Ratner Journal: Acta Neuropathol Date: 2012-10-26 Impact factor: 17.088