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Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor. We retrospectively analyzed the clinical, radiological and histopathological findings as well as follow-up data of two patients with intraventricular liponeurocytomas in Beijing Tiantan Hospital between July 2000 and July 2016. The main clinical manifestations of the two patients were headache. The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on CT scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI). The plaque-like hypodense on CT images and hyperintensity on T1WI resembling fat could be seen inside the tumor. The liponeurocytoma located in the fourth ventricle showed isointensity on T1 and T2WI as well as slight enhancement on contrast. Two patients accepted gross total resection of tumors. Two intraventricular tumors demonstrated similarly histopathological features, such as isomorphic small tumor cells with clear cytoplasm, sheets of monomorphic round cells and focal lipomatous differentiation. In addition, expression of synaptophysin, neuron specific enolase, microtubule-associated protein 2 and S-100 were found. No radiological or clinical evidence of recurrence of the tumors was observed in their follow-up surveys. In conclusion, intraventricular liponeurocytoma has a favorable clinical course, radiological features may be useful in the diagnosis of this rare tumor before surgery.

Introduction Cerebellar liponeurocytoma is a rare tumor of the central nervous system occurring mainly in the posterior fossa, which shows neuronal and variable astrocytic differentiation, along with foci of lipomatous differentiation. Case presentation Herein, we describe a 50-year-old female patient who presented to the hospital complaining of headache, tinnitus, and vertigo with positive cerebellar signs. MRI revealed a left cerebellar tumor. After tumor resection, histological examination and immunohistochemistry were done and the diagnosis of cerebellar liponeurocytoma was confirmed. Discussion Liponeurocytoma may be mistaken as a medulloblastoma with lipidized cells or a lipomatous ependymoma. Histopathological examination, reinforced by immunohistochemistry and electron microscopy, are required to distinguish between these entities. 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. Conclusion Liponeurocytoma is a rare benign tumor with cerebellum is the typical site for it. Although surgery is the treatment of choice; however, postoperative radiotherapy may have a role in case of incomplete tumor resection or recurrence., Highlights • Cerebellar liponeurocytoma is a rare benign tumor that mainly occurs in the posterior fossa. • Surgery is the first line in management. • Postoperative radiotherapy has a role in case of incomplete tumor resection or 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.

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.

INTRODUCTION Classified as a benign glioneural tumor, cerebellar liponeurocytoma is a rare disease (fewer than 80 reported cases) and was upgraded from WHO grade I to grade II in 2007 due to its high recurrence rate. The authors report a case of definitive radiation therapy for recurrent subtotally resected cerebellar liponeurocytoma. METHODS An 80-year-old man having undergone seven resections for his left cerebellar liponeurocytoma without adjuvant therapy over the previous decade at outside institutions was referred for radiation therapy two months following his eighth resection, where gross total resection was limited by the lesion proximity to his brainstem resulting in a 2 cm residual left cerebellar lesion. Pathology revealed tumor cells strongly positive for synaptophysin and a Ki-67 labeling index < 1%. Due to the propensity of this disease to recur following resection, his entire resection cavity was treated with external beam radiation therapy (EBRT) to 46 Gy, followed by a 10 Gy boost to his residual disease yielding a total of 56 Gy to the residual disease. RESULTS Reimaging following the initial 46 Gy revealed the residual disease remained amenable (< 3 cm) to stereotactic radiosurgery (SRS), which was delivered via linear accelerator (10 Gy to the 80% isodose line) in a single fraction. Following EBRT + SRS, the patient responded well. At last follow-up, he has demonstrated no evidence of disease progression, brainstem-related morbidity or surgical incision-related morbidity. CONCLUSIONS The first reported case of SRS treatment of cerebellar liponeurocytoma as the culmination of a coordinated definitive plan beginning with EBRT supports the applicability and feasibility of this treatment strategy following subtotal resection. This case indicates that a radiation treatment plan similar to that for central neurocytoma may be an optimal strategy, and suggests that adjuvant radiation therapy following operative resection of this rare disease may be underutilized.

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.

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.

Cerebellar liponeurocytoma (CL) described under many different names, is a rare WHO grade I or II well differentiated neurocytic tumor of the cerebellum with focal lipomatous differentiation. Mainly reported in adulthood, it is fought to be a posterior fossa benign tumor. In this paper, we talk about a 64-year-old woman, following up for Parkinson’s disease presented in our department for 7 months’ history of headache and gait disturbance. MRI showed a right cerebellopontine angle (CPA) heterogeneous unlimited tumor mimicking an epidermoid cyst. She underwent a lateral suboccipital craniectomy procedure that permitted obtaining the whole gross total resection of this tumor. The histopathological diagnosis was a cerebellar liponeurocytoma. She was discharged from hospital the 8th day after surgery and was free of symptoms since 5 years.

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.

Cerebellar liponeurocytoma is a rare benign neuroepithelial tumour. We describe the case of a 50-year-old man presenting with signs of increased intracranial pressure and cerebellar dysfunction. Magnetic resonance imaging showed a heterogeneous, well-circumscribed cerebellar mass with a predominant adipose content. Diffusion-weighted imaging showed an isointense mass with a hyperintense rim. Craniotomy demonstrated a soft grey mass with intratumoral bright patchy yellow areas. Histological and immunohistochemical findings indicated an advanced neuronal, glial and focal lipomatous differentiation with a low level of mitotic activity.

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 rare and newly identified neoplasm found in adults which is reputed to be benign. Its salient morphological characteristics are advanced neuronal/neurocytic differentiation, the presence of lipomatous areas, low mitotic rate, and the absence of necrosis, pleomorphism and vascular hyperplasia. Reported is a case of relapsing liponeurocytoma which occurred 3 and a half years after the radical excision of the primary lesion. Histopathological aggressive features (mitoses and a high proliferation index as evaluated by MIB-1) were shown in the primary lesion and recurrence of the tumor. We suggest that liponeurocytoma is an uncertain malignant potential lesion when mitoses are present and the MIB-1 positive cells are more than 10%.

Cerebellar liponeurocytoma that has been recently identified as a distinct entity by the World Health Organization is characterized by areas of lipomatous differentiation and apparently by a favorable prognosis. In this paper, we described a case of 49-year-old female showing progressive clinical course inspite of a low labeling/mitotic index. We also review the relevant literature. Although, basically all reported cases share a similar histological pattern, i.e. focal accumulations of adiposities in an otherwise typical small cell tumor like central neurocytoma, some clinical properties such as (age, proliferative potential, therapy and survival) are not uniform. The exact biological behavior of this special variant tumor is established. Yet, this needs further confirmation on a large number of cases with longer follow-up periods.

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.

Resumen Presentamos en nuestro trabajo, un caso de liponeurocitoma cerebeloso, tumor poco frecuente, observado en una mujer de 62 anos de edad. Dicho tumor mostro una imagen histologica compatible con un tumor de celulas pequenas, de escasa agresividad, positivo para marcadores neuronales (sinaptofisina y enolasa neuronal especifica) con areas focales de diferenciacion lipidica. Tras establecer un diagnostico diferencial con otras neoplasias, comentamos brevemente las caracteristicas de esta rara entidad.

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.

A 54-year-old female presented with a two year history of progressive headaches and upper neck pain. The headaches were worse with coughing and bending. Neurological examination was unremarkable including a normal cranial nerve examination. There was no papilloedema. A computed tomogram (CT) demonstrated a midline, posterior fossa, partly fatty, partly solid mass (Figure 1). Magnetic resonance imaging (MRI) demonstrated a mixed fatty, solid mass arising from the fourth ventricle and extending downward below the foramen magnum to the C1 level (Figure 2). The solid portions demonstrated enhancement. In addition, in the lateral right cerebellar hemisphere, there was a second, separate, solid, enhancing mass without any connection to the larger central lesion. A subtotal resection of the tumor was achieved through a suboccipital craniectomy.

Cerebellar liponeurocytoma is a rare benign neuroepithelial tumor. We describe the case of a 50- year-old man, presenting with signs of increased intracranial pressure and cerebellar dysfunction. Magnetic Resonance Imaging (MRI) showed a heterogeneous, well-circumscribed cerebellar mass with a predominant adipose content. Diffusion Weighted Imaging (DWI) showed an isointense mass with a hyperintense rim Craniotomy demonstrated a soft, gray mass with intratumoral bright patchy yellow areas. Histological and immunohistochemical findings indicated an advanced neuronal, glial and focal lipomatous differentiation with a low level of mitotic activity.

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.

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. 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. 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.