Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

2.50
Hdl Handle:
http://hdl.handle.net/10541/81196
Title:
Malignant peripheral nerve sheath tumours in neurofibromatosis 1.
Authors:
Evans, D Gareth R; Baser, Michael E; McGaughran, J; Sharif, Saba; Howard, E; Moran, Anthony
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.
Affiliation:
University Department of Medical Genetics, St Mary's Hospital, Manchester M13 0JH, UK. Gevans@central.cmht.nwest.nhs.uk
Citation:
Malignant peripheral nerve sheath tumours in neurofibromatosis 1. 2002, 39 (5):311-4 J. Med. Genet.
Journal:
Journal of Medical Genetics
Issue Date:
May-2002
URI:
http://hdl.handle.net/10541/81196
PubMed ID:
12011145
Type:
Article
Language:
en
ISSN:
1468-6244
Appears in Collections:
All Christie Publications

Full metadata record

DC FieldValue Language
dc.contributor.authorEvans, D Gareth R-
dc.contributor.authorBaser, Michael E-
dc.contributor.authorMcGaughran, J-
dc.contributor.authorSharif, Saba-
dc.contributor.authorHoward, E-
dc.contributor.authorMoran, Anthony-
dc.date.accessioned2009-09-15T17:23:08Z-
dc.date.available2009-09-15T17:23:08Z-
dc.date.issued2002-05-
dc.identifier.citationMalignant peripheral nerve sheath tumours in neurofibromatosis 1. 2002, 39 (5):311-4 J. Med. Genet.en
dc.identifier.issn1468-6244-
dc.identifier.pmid12011145-
dc.identifier.urihttp://hdl.handle.net/10541/81196-
dc.description.abstractBACKGROUND: 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.en
dc.language.isoenen
dc.subject.meshAdolescent-
dc.subject.meshAdult-
dc.subject.meshAged-
dc.subject.meshEngland-
dc.subject.meshFemale-
dc.subject.meshHumans-
dc.subject.meshIncidence-
dc.subject.meshLongitudinal Studies-
dc.subject.meshMale-
dc.subject.meshMiddle Aged-
dc.subject.meshNeurofibromatosis 1-
dc.subject.meshRisk Assessment-
dc.subject.meshSurvival Rate-
dc.titleMalignant peripheral nerve sheath tumours in neurofibromatosis 1.en
dc.typeArticleen
dc.contributor.departmentUniversity Department of Medical Genetics, St Mary's Hospital, Manchester M13 0JH, UK. Gevans@central.cmht.nwest.nhs.uken
dc.identifier.journalJournal of Medical Geneticsen

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