A multi-institutional study of brainstem gliomas in children with neurofibromatosis type 1.

ABSTRACT:

Objective

To define the clinical and radiologic features of brainstem gliomas (BSGs) in children with neurofibromatosis type 1 (NF1).

Methods

We performed a retrospective cross-sectional study of 133 children with NF1 and concurrent BSGs cared for at 4 NF1 referral centers. BSG was determined using radiographic criteria. Age at diagnosis, tumor location and appearance, clinical symptoms, treatment, and presence of a concurrent optic pathway glioma were assessed.

Results

The average age at BSG diagnosis was 7.2 years, and tumors occurred most often in the midbrain and medulla (66%). The majority of children with NF1-BSGs were asymptomatic (54%) and were not treated (88%). Only 9 of the 72 asymptomatic children received treatment because of progressive tumor enlargement

SUBMITTER: Mahdi J 

PROVIDER: S-EPMC5395076 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature