Brainstem glioma presenting as pruritus in children with neurofibromatosis-1

Abstract

Children with neurofibromatosis type 1 (NF1) are at increased risk of developing tumors of the nervous system. The most common intracranial tumors are low-grade astrocytomas, which most frequently involve the optic pathway, and less often, the brainstem. In cases of brainstem glioma in NF1, treatment decisions are frequently complicated by the paucity of symptoms referable to the tumor. Here, we describe 2 children with NF1 whose initial presentation of a growing brainstem glioma was localized pruritus. This unusual presentation emphasizes the importance of appreciating subtle, often hard to localize, symptoms in this high-risk population.