Risk factors for treatment-refractory and relapsed optic pathway glioma in children with neurofibromatosis type 1.

ABSTRACT:

Background

Nearly one-third of patients with neurofibromatosis type 1-associated optic pathway glioma (NF1-OPG) fail frontline chemotherapy; however, little is known about risk factors for treatment failure.

Methods

We performed a retrospective multi-institutional cohort study to identify baseline risk factors for treatment-refractory/relapsed disease and poor visual outcome in children with NF1-OPG. Refractory/relapsed NF1-OPG was defined as a requirement of two or more treatment regimens due to progression or relapse.

Results

Of 111 subjects eligible for inclusion, adequate clinical and visual data were available for 103 subjects from 7 institutions. Median follow-up from the initiation of first chemotherapy regimen was 95 months (range 13-185). Eighty-four (82%) su

SUBMITTER: Kotch C 

PROVIDER: S-EPMC9340646 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature