Cerebellar liponeurocytoma is a rare, low-grade neoplasm (WHO grade 2) characterized by neuronal or neurocytic differentiation and variable glial differentiation with lipoma-like alterations. First described in 1978 and recognized in the WHO classification in 2000, it presents significant challenges in radiological and pathological differentiation. This differentiation is crucial to avoid unnecessary or deleterious treatments. The patient presented with symptoms including headache, dizziness, hiccups, nausea, vomiting, tinnitus, and left-sided ataxia. Imaging revealed a heterogeneous lesion in the left cerebellopontine angle. Resection was performed via a left suboccipital retrosigmoid approach, resulting in significant improvement in axial and appendicular ataxia, although left-sided anacusis persisted. Histopathological analysis confirmed cerebellar liponeurocytoma showing hypercellularity, with neurocytic cells containing lipid accumulations. Immunohistochemical analysis revealed diffuse expression of synaptophysin and focal expression of glial fibrillary acidic protein, with a Ki-67 proliferation index of 7%. Cerebellar liponeurocytoma is a rare tumor requiring accurate histopathological differentiation to determine the appropriate treatment. Resection remains the mainstay of treatment, with postoperative radiotherapy potentially reducing recurrence. Multidisciplinary follow-up is crucial for managing residual symptoms and monitoring for recurrence. This case aligns with existing literature and underscores the importance of comprehensive diagnostic and therapeutic approaches to improve patient outcomes. https://thejns.org/doi/10.3171/CASE24521.

Cerebellar liponeurocytoma (CL) is a rare WHO grade 2 tumor characterized by advanced neuronal differentiation and variable lipomatous features. Initially classified as a subtype of medulloblastoma, CL was later considered as a distinct entity owing to its peculiar morphological and molecular features and significant better outcome. Typically affecting adults, CL often presents with symptoms related to cerebellar dysfunction, including headaches, ataxia, and gait disturbances. On magnetic resonance imaging, this tumor presents as a well-defined, heterogeneous mass with lipomatous components, which may be less or more apparent depending on their extent. Histologically, CL is composed of neurocytic cells and lipidized tumor cells; the immunohistochemical positivity for synaptophysin and NeuN confirms the neuronal differentiation of neoplastic cells. In spite of its morphological similarity to medulloblastoma, CL lacks the genetic alterations commonly found in this tumor, but some cases display TP53 mutations. Complete surgical resection is the gold standard treatment, whereas the benefit of adjuvant radiotherapy is controversial. CL generally harbors a favorable prognosis, with low recurrence rates in cases with incomplete resection or high proliferative index. The present paper comprehensively reviews the literature about CL, emphasizing the clinicopathologic and molecular features of this unusual but distinct neuropathological entity.

Cerebellar liponeurocytoma is a rare WHO grade II glioneuronal tumor, characterized by a benign course and a more favorable prognosis. In this paper, we report a 52-year-old man, hospitalized in emergency because of clinical signs of increased intracranial pressure, and gait disturbances. The MRI revealed a lateral well shaped solid lesion within the left cerebellar hemisphere, having a low signal intensity compared to the cerebrospinal fluid, and causing a triventicular hydrocephalus and tonsillar herniation. The patient underwent a direct approach of the tumor through a left suboccipital craniectomy. Total removal of the lesion was achieved, and the histological diagnosis was cerebellar liponeurocytoma. The tumor cell proliferation index was < 6 %, therefore, radiotherapy and chemotherapy were not implemented after surgery. After 5 years of close follow-up there were no clinical or radiological signs of recurrence. Our objective in reporting another case of this unusual tumor is to discuss clinical profile, radiologic and histologic features as well as treatment and prognosis of this rare posterior fossa tumor.