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Cerebellar liponeurocytoma is a rare clinicopathological entity, included in the 2000 World Health Organization (WHO) classification of tumors of the central nervous system in the category of glioneuronal tumors. These are WHO Grade II, slow-growing tumors in adults with known recurrences. To date, about 70 cases of liponeurocytomas have been reported. This tumor needs to be differentiated from oligodendroglioma as well as medulloblastoma which are more common and aggressive, requiring postoperative chemoradiation. A high index of suspicion is thus advocated for the diagnosis. We present one such case of this rare entity reported in our institute. A 42-year-old female presented with a 4-month history of headache, neck pain, reduced vision, and cerebellar signs. Imaging showed a midline lesion involving the cerebellar vermis and left lobe suggestive of anaplastic ependymoma/hemangioblastoma. The final diagnosis of cerebellar liponeurocytoma was rendered on routine histopathological examination supported by immunohistochemical analysis.

Cerebellar liponeurocytoma or lipomatous medulloblastoma is a rare oncological entity. Knowledge regarding the management and outcomes of these rare tumors are still evolving. Very few cases have been described previously in the literature. The authors report a case of a middle-aged woman operated on twice, 8 years apart, with uneventful postoperative follow-ups. Radiological characteristics were revealed atypically on the computed tomography scan and magnetic resonance imaging. Histopathological study supported a cerebellar liponeurocytoma with classic immunohistochemical features. Through this report, the authors aim to describe atypical radiological and histopathological features of this rare entity with good outcome by going through a comprehensive review of the existing literature.

BackgroundCerebellar liponeurocytoma is a rare benign neoplasm of the central nervous system, which arises mainly in adult patients with only 3 cases reported in children. Due to its rarity, the diagnosis and treatment strategies for cerebellar liponeurocytoma remain unclear. The purpose of this study was to explore the epidemiology, clinical features, imaging findings, pathological characteristics, different diagnoses, treatment, and prognosis of cerebellar liponeurocytoma in juveniles.Case DescriptionA 5-year-old boy was admitted to the department of neurosurgery due to a 5-month history of headaches, nausea, vomiting, dizziness, dysphoria, as well as visual blurring associated with the peak of the headache. Magnetic resonance imaging showed a 4.9×5.4×6.2 cm mass located in the fourth ventricle and cerebellar vermis combined with hydrocephalus and periventricular edema. The mass was completely removed, and pathological examination indicated a cerebellar liponeurocytoma of the World Health Organization Grade II classification.ConclusionThe present study was the first to report a cerebellar liponeurocytoma with total tumor resection and adjuvant radiotherapy in a pediatric patient. Total tumor resection and postoperative radiotherapy together with close and long-term follow-up seem to be the optimal treatment strategy for juvenile patients. However, the side-effect of radiation needs to be considered.

Abstract Background Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation with foci of lipomatous differentiation. Liponeurocytoma develops in adult patients and is defined in the World Health Organization classification of 2016 as a rare benign grade II tumor. Case presentation A 39-year-old Italian man presented to our department suffering from headache and nausea. Magnetic resonance imaging revealed a right-sided cerebellar lesion showing poor contrast enhancement without an obstructive hydrocephalus. Surgery was indicated and total tumor resection was achieved. He was discharged without any neurological deficits. Histopathological examinations revealed a cerebellar liponeurocytoma. A neurological follow-up examination revealed no neurological deficit directly after surgery and 1 year later. Radiotherapy was recommended at the neurooncological board despite the total removal of the tumor, but our patient refused adjuvant radiotherapy. Magnetic resonance imaging of his neurocranium with and without contrast enhancement 48 hours after surgery and 15 months after surgery showed no residual tumor. Conclusions Liponeurocytomas are rare benign tumors occurring in the majority of cases in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy has to be discussed individually, but seems to be sufficient if complete tumor resection is not achieved or in cases of a tumor recurrence.

We present a case of a rare neoplasm in a 77-year-old woman with previous oncological history, who developed a rapid onset of cerebellar symptoms. The neuroimaging detected a posterior fossa tumor suspected of meningioma which was completely resected soon after. Histologically the neoplasm had two components with different immunophenotype. One constituent was lobular, composed of monotonous mitotically active round cells with a predominant neuronal profile. The second, astrocytic component contained lipomatous cells intermixed with larger gemistocytic astrocytes. Fields of geographic necrosis as well as multifocal microvascular proliferation were observed. The Ki67 proliferation index was 12%. After two years of follow-up the patient remains free of symptoms and radiologic recurrence. The presented case of cerebellar liponeurocytoma is unusual in terms of its atypical histological features and prominent astrocytic component. The authors propose that the term ‘lipomatous glioneuronal tumor’ seems to be more appropriate for this type of lesion, considering its histologic spectrum and possible extracerebellar location.

Liponeurocytoma is a newly defined clinical entity predominantly seen in the cerebellum as a slow-growing tumor. In this report, we present the case of a 6-year-old Nigerian girl with a liponeurocytoma, and review of literature.

Cerebellar liponeurocytoma consists of well-differentiated neurons with the cytology of neurocytes in addition to a population of lipidized cells. Hence it is biphasic in appearance and has been included in the category of glioneuronal tumors of the central nervous system by the WHO working group on the Classification of Tumors of the Nervous System. However, liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification, nine cases with similar histological and immunohistochemical features have also been described in the lateral ventricles. We describe here such a lateral ventricular tumour in a 30-year-old woman, characteristically showing divergent glio-neuronal differentiation and lipidized neoplastic cells. Therefore, we suggest that future WHO tumor classification should consider that liponeurocytomas are not entirely restricted to the cerebellum and henceforth change of nomenclature might be considered, as also pointed out by other authors.