Association of plexiform neurofibroma volume changes and development of clinical morbidities in neurofibromatosis 1.

ABSTRACT:

Background

Plexiform neurofibromas (PN) in neurofibromatosis 1 (NF1) can cause substantial morbidities. Clinical trials targeting PN have recently described decreases in PN volumes. However, no previous study has assessed the association between changes in PN volumes and PN-related morbidities. Our objective was to assess if increasing PN volume in NF1 is associated with increasing PN-related morbidity.

Methods

This is a retrospective review of patients enrolled on the NCI NF1 natural history study with ≥7 years of data available. Morbidities including pain, motor dysfunction, vision loss, and PN-related surgery were assessed at time of baseline PN MRI with volumetric analysis and time of MRI with maximum PN volume.

Results

Forty-one patients (median age at baseline 8

SUBMITTER: Gross AM 

PROVIDER: S-EPMC6231202 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature