Cerebellar Liponeurocytoma: Case report of a rare tumor and a brief review of the literature

Cerebellar liponeurocytoma (cLNC) is a rare WHO (World Health Organization) Grade II neuronal tumor characterized by neurocytic differentiation with lipomatous components and typically occurring in the posterior fossa of adults. We present a case of a 51-year-old female with a six-month history of progressive gait instability and incoordination, and subsequently developed morning headaches, nausea, vomiting, and dysarthria. She had signs of increased intracranial pressure. Neurological exams showed truncal ataxia, bilateral dysmetria, dysdiadochokinesia, and papilledema. Her MRI showed a 4.5 x 3.8 x 4.2 cm well-circumscribed, heterogeneous mass located in the right cerebellar hemisphere. Areas of T1 hyperintensity suggestive of fat were observed, accompanied by mild heterogeneous enhancement. The mass compressed the fourth ventricle, resulting in early hydrocephalus. She underwent suboccipital craniotomy with gross total tumor resection. Her surgical pathology revealed a tumor volume of approximately 35% lipomatous component, and low mitotic activity was noted in the uniform round neurocytic cells. Immunohistochemistry (IHC) studies revealed strong positivity for synaptophysin, NeuN, and NSE, with a low Ki-67 of 4%, and thus, the diagnosis of cerebellar liponeurocytoma was confirmed. Three years postoperatively, the patient had notable neurological improvement, complete resolution of intracranial hypertension, and no signs of recurrence. This case illustrates the need to include cLNC in the differential diagnosis of fat-containing posterior fossa masses in adults and emphasizes the improved prognosis with gross total resection.