Cerebellar gliomas in children with NF1: pathology and surgery

Abstract 

Cerebellar gliomas associated with NF1 (CGNF1) are rarely reported in the literature, and they are considered to be malignant in a high proportion of cases. In an attempt to improve the definition of this disease and clarify its management, we reviewed our patients with CGNF1 and compared their tumors with sporadic cerebellar gliomas (SGC). We operated on six children with CGNF1, all but one of whom were asymptomatic. They represented one-tenth of all pediatric cerebellar gliomas, and one third of NF1- associated gliomas seen in our institution. CGNF1 appeared at a later age than SCG. They are seated near the roof of the IV ventricle and are not related to white matter hypersignal hamartomas. Most of these tumors showed radiological progression. They were four pilocytic astrocytomas, one ganglioglioma, and one malignant astrocytoma. One patient had tumor recurrence after 8 years, and the others are still disease free. The overall outcome appeared to be better for GCNF1 than for SCG. On account of the regular growth, uncertain pathology, and good surgical outcome, we advocate systematic resection of these tumors.