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This article discusses the case of a 31-year-old woman who presented with symptoms of headache, dizziness, and phosphenes. Magnetic resonance imaging (MRI) revealed a well-circumscribed mass in the left cerebellar hemisphere. The tumor was diagnosed as a cerebellar liponeurocytoma, a rare and slow-growing tumor that occurs in the cerebellum. The article highlights the diagnostic challenges of distinguishing cerebellar liponeurocytoma from other tumors, such as medulloblastoma, and emphasizes the importance of thorough examination and analysis of histological and immunohistochemical features for accurate diagnosis. The study concludes that cerebellar liponeurocytoma has distinct genetic and epigenetic features that can aid in diagnosis, and that the identification of lipomatous foci is crucial for distinguishing this tumor from other similar tumors. [Extracted from the article]

The literature on cerebellar liponeurocytoma (CL) has never been systematically assessed using bibliometric analytic methodologies. We quantitatively analyzed the major trends and scientific output regarding CL, highlighting potential avenues for research. Elsevier's Scopus database was used to collect all published studies relevant to cerebellar liponeurocytoma from 1978 to 2021. The specific bibliometric parameters were extracted and analyzed with R v4.1.2. Our search yielded 108 documents published in 67 sources from 1978 to 2021. The annual growth rate of publications regarding CL has been 7.47% per year since 1978. Journals with the most publications on CL include Clinical Neuropathology and Neurology India (n = 5), followed by Acta Neuropathologica and Journal of Neuro-oncology (n = 4). A total of 529 authors have published on CL and they have been cited 598 times. The 10 most influential authors in the field were determined using their total number of citations and the local H-index. Kleihues P has the highest number of citations (n = 177) with a local H index of 3, followed by Chimelli L with 167 citations and a local H index of 4. Davis DG has 149 citations and a local H index of 3. China had the most single country publications followed by India, Italy, and the USA. France and Austria have the most multiple country publications followed by China, Tunisia, Brazil, United Kingdom, Egypt, and Israel. Our study is the first bibliometric analysis evaluating the present literature and publication trends in CL. Generally, the current literature has a few studies regarding CL relative to other neuro-oncological pathologies. This can be due to the low incidence of the disease and highlights a need for high volume database studies that can offer high quality evidence on the subject. [ABSTRACT FROM AUTHOR]

Cerebellar liponeurocytoma (CL) is an unusual tumor, histologically composed of a mixture of small to medium‐sized, rounded neurocytic cells and a variable lipomatous component. Although CL was originally considered as a subtype of medulloblastoma, subsequent molecular studies demonstrated that this tumor was a distinct entity, exhibiting the tumor protein p53 gene (TP53) missense mutations in 20% of cases, chromosome 17 deletion, and the absence of mutations in the adenomatous polyposis coli gene (APC), the protein patched homolog gene (PTCH), the kinase insert domain receptor gene (KDR), and the β‐catenin gene (CTNNB). Apart from these molecular features, little is known about the pathogenesis and the genetic landscape of CL to date. In order to characterize the mutational landscape of CL and identify alterations that are driving tumorigenesis, we report a series of three cases, including one recurrent tumor, analysed by next‐generation sequencing (NGS), which identified a total of 22 variants, of which four were missense mutations, nine were synonymous variants, and nine were located on intronic regions. In particular, DNA sequencing identified missense mutations in APC, KDR, and TP53 that could be implicated in promoting tumor progression and angiogenesis of CL. Furthermore, the NGS analysis revealed that recurrent CL did not have additional genetic changes compared with the primary tumor. Moreover, the high frequencies of detected mutations suggested that the identified alterations are germline variants. Indeed, an additional NGS on the genomic DNA obtained from one of the three patients confirmed the presence of the variants in the germline DNA. In conclusion, the obtained data support the hypothesis that CL is a distinct pathological entity that does not show specific somatic alterations driving tumorigenesis. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Cerebellar liponeurocytoma is a rare oncological entity, and the knowledge about the treatment and outcome of these rare tumors is still evolving. Very few cases have been described in literature. We report a middle-aged male who presented with raised intracranial pressure features and gait ataxia. His imaging features revealed classical features of liponeurocytoma in cerebellar vermis, with abundant fat component evident in both computed tomography and magnetic resonance imaging. He underwent resection of the lesion and has been asymptomatic for 4 years. This report describes the classical radiological and immunohistochemical features of this rare entity with favorable outcome and reviews the existing literature. [ABSTRACT FROM AUTHOR]

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. Highlights • cLNCs are rare benign glioneuronal tumor with unknown natural history and long-term outcomes. • Multifocality is exceedingly rare, with only 4 cases reported previously. • The clinical course is usually marred by frequent recurrences. • Surgical excision is considered the main treatment modality, even for recurrent lesions. • The optimal postoperative management and follow-up protocol remain controversial particularly for multifocal lesions. [ABSTRACT FROM AUTHOR]

Cerebellar liponeurocytoma is rare, and the clinical characteristics and treatment strategy remain unclear. In the present study, a case of cerebellar liponeurocytoma was retrospectively reported and a literature review was performed. A 45-year-old female presented due to occipital headaches, exhibiting a hoarse voice and a broad-based gait. Pre-operative magnetic resonance images revealed a lesion occupying the right hemisphere of the cerebellum and the inferior vermis, compressing the medulla oblongata from the right side, and extending through the foramen magnum to the C2 level. A total resection was performed, and pathological analysis of the lesion showed positivity for synaptophysin, S-100 and neuronal nuclear antigen, partial positivity for Olig-2, and negativity for glial fibrillary acidic protein and epithelial membrane antigen. In addition, the Ki-67 index was low (<5%). Thus, a diagnosis of cerebellar liponeurocytoma was determined. Total resection was successful and the patient was followed up closely. A review of the literature showed that cerebellar liponeurocytoma is mainly located in the cerebellum, with rare extra-cerebellar cases. Certain studies have suggested that the tumor may be located supratentorially and subtentorially, and should be renamed as solely liponeurocytoma. Total resection of the tumor contributes to an improved prognosis, while a subtotal resection and high Ki-67 index lead to recurrence. The tumor is similar to a tumor of low malignancy, with long-term recurrence. Radiation is recommended when there is residual tumor, recurrence or when the Ki-67 is high. [ABSTRACT FROM AUTHOR]

The clinical, histopathological, and ultrastructural features of a cerebellar liponeurocytoma are reported. The tumor, a 3-cm mass localized in the right cerebellar hemisphere, was resected from a 61-year-old man clinically presenting with symptoms of intracranial hypertension. The lesion was composed of small, in some areas closely packed, medulloblastoma-like cells with prominent areas of lipidization phenomena. Moreover, mitoses, cellular atypia, and numerous vascular structures were focally observed. Glial and neuronal differentiation was immunohistochemically noted (glial fibrillary acidic protein, synaptophysin, neurofilaments, and neuron-specific enolase positivity). The p53 oncoprotein was detected in the majority of neoplastic cells and a moderate proliferation activity, evaluated by Mib-1 antibody, was focally appreciated. Ultrastructural study did not show evident neuritic processes, synapses, or dense core neuroendocrine granules. This cerebellar tumor previously called lipidized medulloblastoma and recently renamed cerebellar liponeurocytoma is considered an adult neoplasm with excellent prognosis histologically presenting prominent lipidized areas and, at the immunohistochemical and ultrastructural level, both glial and neuronal differentiation. The present study providesthe first description of a less differentiated and histologically more aggressive form of this unusual tumor. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]