Posterior Fossa Dysembryoplastic Neuroepithelial Tumor: A Neuropathological Report

Dysembryoplastic neuroepithelial tumors (DNTs) are rare neoplastic entries of the central nervous system. Conventionally DNTs are with cortical location and predominantly occur in the temporal lobe associated with epilepsy. Subtentorial DNTs are rare entries. Herein we report a case of a two-year-old female with a DNT located in the cerebellum. The patient presented clinically with new onset gait instability, headaches and strabismus. Neuroradiology revealed a heterogenous, predominantly cystic lesion in the cerebellar vermis and left cerebellar hemisphere, which was interpreted as possible medulloblastoma based on the patient profile. Frozen section neuropathology was more suggestive of a low-grade glial tumor, with conventional histology and immunohistochemistry showing an admixture of glial and neuronal cells - a complex variety of DNT. Due to the histological appearance, differential diagnosis was required with other neuroglial tumors native to the posterior fossa, such as Lhermitte-Duclos disease. There have been several such published case reports, which, although of older patients, present with similar symptoms and neuropathological findings. The complexity of the neuropathological finding in posterior fossa DNTs can lead to future separation of this entry from conventional DNT, as was seen in the past with septum pellucidum DNT, now referred to as myxoid glioneuronal tumor.