Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

DDGR Evans; M Baser; J McGaughran; S Sharif; E Howard; A. Moran

Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

DDGR Evans, M Baser, J McGaughran, S Sharif, E Howard, A. Moran

Research output: Contribution to journal › Article › peer-review

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

Original language	English
Pages (from-to)	311-4
Journal	J Med Genet
Volume	39( 5)
Publication status	Published - May 2002

Keywords

Adolescent
Adult
Aged
epidemiology: England
Female
Humans
Incidence
Longitudinal Studies
Male
Middle Aged
diagnosis: Neurofibromatosis 1
Risk Assessment
Survival Rate

Cite this

@article{16a91511c98646cb859bc1e851894b76,

title = "Malignant peripheral nerve sheath tumours in neurofibromatosis 1.",

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",

keywords = "Adolescent, Adult, Aged, epidemiology: England, Female, Humans, Incidence, Longitudinal Studies, Male, Middle Aged, diagnosis: Neurofibromatosis 1, Risk Assessment, Survival Rate",

author = "DDGR Evans and M Baser and J McGaughran and S Sharif and E Howard and A. Moran",

year = "2002",

month = may,

language = "English",

volume = "39( 5)",

pages = "311--4",

journal = "J Med Genet",

issn = "1468-6244",

publisher = "BMJ ",

}

TY - JOUR

T1 - Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

AU - Evans, DDGR

AU - Baser, M

AU - McGaughran, J

AU - Sharif, S

AU - Howard, E

AU - Moran, A.

PY - 2002/5

Y1 - 2002/5

N2 - 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

AB - 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

KW - Adolescent

KW - Adult

KW - Aged

KW - epidemiology: England

KW - Female

KW - Humans

KW - Incidence

KW - Longitudinal Studies

KW - Male

KW - Middle Aged

KW - diagnosis: Neurofibromatosis 1

KW - Risk Assessment

KW - Survival Rate

M3 - Article

SN - 1468-6244

VL - 39( 5)

SP - 311

EP - 314

JO - J Med Genet

JF - J Med Genet

ER -

Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

Abstract

Keywords

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