Extraventricular neurocytoma in neurofibromatosis Type 1

Abstract

Neurocytomas are rare tumors of the central nervous system that are typically located in the ventricular system. The authors report a case of a child with neurofibromatosis Type 1 (NF1) who had a tumor of the optic nerves and chiasm with signal abnormality extending through the diencephalon, as well as an occipital lobe mass, which was presumed to be part of the visual pathway neoplasm. Because the occipital lobe lesion slowly increased in size over time, while the other areas remained stable, a biopsy was performed. Pathological evaluation revealed an extraventricular neurocytoma of extraventricular neurocytoma. To the authors' knowledge, neurocytomas have not been previously reported in patients with NF1. Because visual pathway gliomas are extremely common in children with NF1, they are often treated empirically as low-grade gliomas without histological confirmation. The importance of obtaining a biopsy in lesions that have atypical imaging features is highlighted.