Cerebellar gliomas in children with NF1: pathology and surgery

Cerebellar gliomas associated with NF1 (CGNF1) are rarely reported in the l iterature, 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 manage ment, we reviewed our patients with CGNF1 and compared their tum ors with sporadic cerebellar gliomas (SGC). We operated on six children wit h CGNF1, all but one of whom were asymptomatic. They represented one-tenth of all pediatric cerebellar gliomas, and one third of NF1-associated glioma s 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 matt er hypersignal hamartomas. Most of these tumors showed radiological progres sion. They were four pilocytic astrocytomas, one ganglioglioma, and one mal ignant astrocytoma. One patient had tumor recurrence after 8 years, and the others are still disease free. The overall outcome appeared to be better f or GCNF1 than for SCG. On account of the regular growth, uncertain patholog y, and good surgical outcome, we advocate systematic resection of these tum ors.