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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.

The biological origin of cerebellar liponeurocytomas is unknown, and hereditary forms of this disease have not been described. Here, the authors present clinical and histopathological findings of a young patient with a cerebellar liponeurocytoma who had multiple immediate family members who harbored similar intracranial tumors. A 37-year-old otherwise healthy woman presented with a history of progressive headaches. Lipomatous medulloblastoma had been diagnosed previously in her mother and maternal grandfather, and her maternal uncle had a supratentorial liponeurocytoma. MRI revealed a large, poorly enhancing, lipomatous mass emanating from the superior vermis that produced marked compression of posterior fossa structures. An uncomplicated supracerebellar infratentorial approach was used to resect the lesion. Genetic and histopathological analyses of the lesion revealed neuronal, glial, and lipomatous differentiation and confirmed the diagnosis of cerebellar liponeurocytoma. A comparison of the tumors resected from the patient and, 22 years previously, her mother revealed similar features. Cerebellar liponeurocytoma is a poorly understood entity. This report provides novel evidence of an inheritable predisposition for tumor development. Accurate diagnosis and reporting of clinical outcomes and associated genetic and histopathological changes are necessary for guiding prognosis and developing recommendations for patient care.

Background Cerebellar liponeurocytoma (cLNC) is a rare benign glioneuronal tumor with only ~60 cases reported since its first description in 1978. They have occurred largely as sporadic cases; however, familial cases with possible autosomal dominant inheritance have also been reported. Surgical excision has been considered the main modality of treatment, even for recurrent lesions. Uncertainties exist regarding the natural history, long-term outcomes, and optimal postoperative management and follow-up protocols for cLNC in general and multifocal cLNC in particular. Multifocality is exceedingly rare, with only 4 cases reported previously. Case Description We present a case of multifocal cLNC in a young woman who had presented with progressive cerebellar symptoms of 5 months’ duration. Her diagnostic neuroimaging evaluations revealed 2 asymmetric mass lesions (1 on each cerebellar hemisphere). We excised the larger lesion to relieve the mass effect and decided to monitor the smaller lesion radiologically. However, she developed a new lesion during the follow-up period and required adjuvant radiotherapy. Conclusion Surgical excision forms the cornerstone of management of cLNCs. However, it is necessary to vigilantly monitor these patients owing to the high recurrence rates of cLNC despite the innocuous histologic features.

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. OA Förderung 2018 - CA Gembruch

Cerebellar liponeurocytoma, first recognized as a distinct entity in the revised WHO classification of Tumors of the Central Nervous System in 2000, is a rare tumor with characteristic histological features and predominant location in the cerebellum. The proliferative index is usually low, and previous reports supported a favorable prognosis. We report a case of a second recurrence of a cerebellar liponeurocytoma with increased proliferative and mitotic activity in which extensive immunohistochemical characterization and genetic profiling were performed. The tumor specimen was characterized in terms of genetic changes frequently associated with gliomas and medulloblastomas. Considering the low number of reported cases, the prognosis of cerebellar liponeurocytoma seems difficult to assess. Our case suggests the existence of different histological grades of cerebellar liponeurocytoma and its possible progression towards a dedifferentiated, malignant phenotype, which has not yet been adequately taken into consideration in the current WHO classification.

There is limited data on the genetic origin and natural history of cerebellar liponeurocytoma. To the best of our knowledge there has been only one report of a familial presentation of this rare entity. We report a 72-year-old female with a posterior fossa tumor presenting with progressive cerebellar signs and symptoms. The patient underwent total tumor resection via an uncomplicated sub-occipital craniotomy. Histopathologic examination was diagnostic for cerebellar liponeurocytoma. Her sister was previously treated for a similar tumor. Our report provides further evidence for the possible existence of a hereditary abnormality predisposing afflicted families to cerebellar liponeurocytoma development.

Liponeurocytoma is not exclusive to the cerebellar or fourth ventricular location. Since its inclusion in the central nervous system tumor classification in 2000, six cases with similar radiological, histomorphological and immunohistochemical features have also been described in the lateral ventricles. In the present study, we report clinical, radiological and pathological findings of three supratentorial and one cerebellar liponeurocytoma from our records, evaluated with an extensive panel of immunohistochemistry, and review published cases in the literature. The immunohistochemical pattern of supratentorial and infratentorial liponeurocytomas are almost identical, which indicates that these tumors are homologous.

A 42-year-old man presented with a 3-year history of headache. Neurologic examination was unremarkable. Brain MRI showed multiple peripheral cerebellar nodules, some containing fat (figure). Biopsy revealed neurocytic cancer cells scattered with mature fat tissue. The approach was conservative with serial imaging follow-up. Cerebellar liponeurocytoma is a rare tumor classified as grade II by the World Health Organization,1 now considered a clinicopathologic entity distinct from medulloblastoma.1 It is almost always solitary and almost always cerebellar. The clear multifocality of the MRI findings makes this case unusual.2

Cerebellar liponeurocytoma is a newly recognized, rare clinicopathological entity commonly described in the cerebellar hemispheres or the vermis. We present a rare case of cerebellar liponeurocytoma arising from the left cerebellar amygdala with extracranial extension. Such a condition has never been previously reported.

✓ Cerebellar liponeurocytoma is a rare tumor of the posterior fossa that has many morphological similarities to medulloblastoma and neurocytoma. Recently the World Health Organization working group for classification of central nervous system neoplasms adopted the term “cerebellar liponeurocytoma” to provide a unified nomenclature for a tumor variously labeled in the literature as lipomatous medulloblastoma, lipidized medulloblastoma, medullocytoma, neurolipocytoma, lipomatous glioneurocytoma, and lipidized mature neuroectodermal tumor of the cerebellum. The rarity of this tumor and paucity of pertinent information regarding its biological potential and natural history have resulted in the application of various treatment modalities. It is suggested in the available literature that these lesions have a much more favorable prognosis than typical medulloblastomas, and that adjuvant therapy for liponeurocytoma need not be as extensive as that administered for medulloblastomas.

WOS: 000334570100011, PubMed: 24729347, A rare tumour, cerebellar liponeurocytoma, is classified into glioneuronal tumours under the 2000 World Health Organization (WHO) classification of tumours of the central nervous system. The current 2007 WHO classification, therefore, assigns grade II to the cerebellar liponeurocytoma. Tumours are predominantly localized in cerebellar hemispheres, and the second most common location is the vermis. To date, approximately 40 reported cases of cerebellar and 10 cases of supratentorial introventricular liponeurocytoma have been reported. In this report, an unusual case of cerebellar liponeurocytoma was presented with extracerebellar location. In the future tumour classification, it should be considered that liponeurocytomas are not restricted only to the cerebellum, but they are located in supratentorial areas as well.

Cerebellar liponeurocytoma is a rare tumor recently included by World Health Organization in the classification of brain tumors as a separate clinicopathological entity separate from medulloblastoma. However, because of the rarity of the tumor, the natural history of the tumor is still not yet been defined. We report a patient with cerebellar liponeurocytoma with unusual clinical and pathological aggressive features. This patient suggests the possible existence of different histological grades of liponeurocytoma and its possible progression to a malignant grade.