Disease course of neurofibromatosis type 2: a 30-year follow-up study of 353 patients seen at a single institution.

ABSTRACT:

Background

Limited data exist on the disease course of neurofibromatosis type 2 (NF2) to guide clinical trial design.

Methods

A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990 and 2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred, and inheritance type. Interventions for NF2-related tumors were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death.

Results

Three hundred and fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring, 60 patients (17.0%) died. The annual mean number of patient

SUBMITTER: Forde C 

PROVIDER: S-EPMC8248850 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature