RESUMEN
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.
RESUMEN
In this case report, we characterize an instance of diagnosis, treatment, characteristics, and outcomes of a patient with a liponeurocytoma, a rare WHO grade II brain tumor first described in 1978. This tumor has been described with a wide array of radiographic, microscopic, and histologic features, and there remains no consensus regarding the role of radiation therapy. Most patients have favorable outcomes after surgical resection. Here we present the case of a 46-year-old female who underwent suboccipital craniectomy for resection of a cerebellar mass, which was diagnosed as liponeurocytoma on final pathology. The patient experienced resolution of symptoms and is neurologically intact two years after resection of the tumor.
RESUMEN
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
Asunto(s)
Bibliometría , Humanos , Francia , Reino Unido , Italia , EgiptoRESUMEN
Background: Liponeurocytomas are rare neurocytic neoplasms that most often arise in the posterior fossa and affect individuals in the third and fifth decades of life. Most reported cases of this unique tumor in the literature have described a favorable clinical prognosis without recurrence. However, increasing reports of recurrent cases prompted the World Health Organization, in 2016, to recategorize the tumor from Grade I to the less favorable Grade II classification. We conducted a systematic review to identify recurrent cases of this unique tumor and to summarize differences between the primary and recurrent cases of liponeurocytoma. Methods: A systematic review exploring recurrent liponeurocytoma cases was conducted by searching the PubMed, Google Scholar, and Scopus databases for articles in English. Abstracts from articles were read and selected for full-text review according to a priori criteria. Relevant full-text articles were analyzed for symptoms, imaging, location, histological, pathological, treatment, and recurrence-free time between the primary and recurrent cases. Results: Of 4392 articles, 15 articles accounting for 18 patients were included (level of evidence: IV) in the study. Recurrence-free time decreased from an average of 82 months between the primary tumor resection to first recurrence to 31.3 months between the first and second recurrence. Recurrent tumors demonstrated increased pleomorphic neural cells, necrosis, vascular proliferation, and MIB-1 index when compared to the primary tumor. Several cases also demonstrated decreased lipidizing components when compared to the primary tumor, further indicating increased dedifferentiation. The primary treatment for this tumor was surgical resection with occasional adjunctive radiotherapy. Conclusion: Recurrent cases of liponeurocytoma have features of increased malignant proliferation compared to the primary cases. The standard treatment for these primary and recurrent tumors is gross total resection. The role of adjunctive radiotherapy remains a matter of debate.
RESUMEN
BACKGROUND: Cerebellar liponeurocytoma is rare intracranial tumor appearing mostly in the posterior fossa. CASE DESCRIPTION: We hereby report a long follow-up of a case of cerebellar liponeurocytoma in a 60-year-old female. At first, she presented in March of 2010 with the symptoms of hydrocephalus and was found to have a lesion located in the fourth ventricle. The tumor was resected with a small remnant around the brainstem which grew on serial imaging. Due to slow tumor growth, the patient was treated with conformal radiotherapy and was kept under follow-up with both outpatient visits and serial brain imaging. In 2018, due to low back pain and lumbar radicular pain, a new set of images of the spine was obtained which revealed multilevel intradural tumor spinal dissemination. The patient further underwent an open spinal biopsy at the level of L5 which revealed the same pathology of the intracranial tumor. The patient went on to receive total spine irradiation. CONCLUSION: This case report describes a rare metastatic phenomenon to the spinal cord of the exact same pathology and grade of an intracranial cerebellar liponeurocytoma tumor.
RESUMEN
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.
RESUMEN
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 ß-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.
Asunto(s)
Poliposis Adenomatosa del Colon , Neoplasias Cerebelosas , Meduloblastoma , Poliposis Adenomatosa del Colon/genética , Carcinogénesis , Neoplasias Cerebelosas/genética , Humanos , Meduloblastoma/patología , MutaciónRESUMEN
BACKGROUND: Cerebellar 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 DESCRIPTION: A 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. CONCLUSION: The 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.
RESUMEN
Background: Cerebellar liponeurocytoma is an extremely rare benign tumor which generally occurs in cerebellum and is almost always solitary. Multifocal cerebellar liponeurocytoma is exceedingly rare, only 8 cases has been reported so far. Herein we present the 9th case of multifocal cerebellar liponeurocytoma in a 70-year-old woman with the complete clinical course and comprehensive imaging findings. Case Presentation: A 70-year-old woman presented with a history of intermittent headache for 5 years. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain have been performed and suggested a diagnosis of teratoma based on the imaging findings. After the surgical resection of the lesion, histopathological and immunohistochemical analyses revealed neuronal, glial, and lipomatous components and confirmed the diagnosis of multifocal cerebellar liponeurocytoma after surgical resection. During the 2-year follow-up period, the patient showed no signs of recurrence or metastasis. Conclusion: We described the radiological characteristics and clinical course of an exceedingly rare case of multifocal cerebellar liponeurocytoma in the cerebellar vermis and temporal lobe. The clear multifocality makes this case unusual.
RESUMEN
Cerebellar liponeurocytoma (cLNC), categorized as a World Health Organization grade II tumor, is a rare neoplasm characterized by advanced neuronal/neurocytic differentiation and focal lipid accumulation in neuroepithelial tumor cells. However, the expression and genetic profiling of cLNC have been poorly studied. A 44-year-old woman with a three-year history of cerebellar ataxia and numbness in lower extremities underwent radiological examination revealing multiple contrast-enhancing tumors at the floor of the fourth ventricle and in the lower vermis, and spinal dissemination. The high uptake of 11 C-methionine in positron emission tomography (Met-PET) supported the preoperative cLNC diagnosis. Subtotal removal of the tumor around the obex and inferior vermis was performed. Histologically, the tumor was composed of small, uniform cells with round nuclei in a sheet-like fashion. Tumor cells were diffusely reactive for the neuronal markers synaptophysin and neurofilament. Vacuolate cells with a displacement of nuclei suggested the accumulation of lipid, which was further supported by immunohistochemical staining of S-100. These findings confirmed the diagnosis of cLNC. Next-generation sequencing of tumoral DNA detected a splice site mutation in the ATRX gene. Further reports of cLNC cases with detailed expression and genetic profiles are essential for precise diagnosis and clarifying the oncogenic pathway in cLNC.
Asunto(s)
Neoplasias Cerebelosas/genética , Neoplasias Cerebelosas/patología , Metabolismo de los Lípidos , Neurocitoma/genética , Neurocitoma/patología , Neoplasias de la Médula Espinal/patología , Médula Espinal/patología , Adulto , Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/metabolismo , Femenino , Humanos , Invasividad Neoplásica , Neurocitoma/diagnóstico por imagen , Neurocitoma/metabolismo , Tomografía de Emisión de Positrones , Proteína Nuclear Ligada al Cromosoma X/genéticaRESUMEN
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.
RESUMEN
BACKGROUND: Liponeurocytoma is an uncommon tumor of the central nervous system. It is very rare for this tumor to originate within the lateral ventricle. In the context of the rarity of this tumor entity, this review article aims to summarize the clinical, radiological, and pathological features of lateral ventricular liponeurocytoma to facilitate its diagnosis and management. METHODS: Here, we conduct a systematic literature review using the Pubmed, Scopus, and Cochrane Library database for all cases of lateral ventricular liponeurocytoma. A case illustration complements this review. RESULTS: The described cases from 1997 onward include 14 cases that have been published in full papers in the English literature. Six additional cases are reported in short English abstracts in full non-English papers, and one case was described in a central neurocytoma report. There is a definite male predominance of 70% (14 male) and a mean age of 37 years (range 24-62). Heterogenous enhancement and signals in magnetic resonant images (MRI) are the radiological characteristics. In all reported cases, the presence of lipocytes and fat vacuoles is considered the paramount histopathological feature. Total surgical resection was achieved in 80% (12 out of 15) of the cases. Only two cases (including ours) received radiation therapy. Recurrence was seen in two patients during follow-up that was treated by radiation therapy in one and surgery in the other. The proliferation index is mostly below 5% in all cases, with the Ki-67 range between<1% to 10%. CONCLUSIONS: Lateral ventricular liponeurocytoma has been treated effectively by surgical resection in a limited number of cases. The decision for radiation therapy is based on a high proliferation index and tumor recurrence.
Asunto(s)
Neoplasias Cerebelosas , Lipoma , Neurocitoma , Adulto , Humanos , Ventrículos Laterales , Lipoma/diagnóstico por imagen , Lipoma/cirugía , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neurocitoma/diagnóstico por imagen , Neurocitoma/cirugía , Adulto JovenRESUMEN
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.
RESUMEN
INTRODUCTION: Liponeurocytomas are mostly localized in cerebellar hemispheres and the second most common location is the vermis. It is rarely observed within the intracranial ventricles. Here, we present a case of liponeurocytoma located in the right lateral ventricle and the systematic review of the literature. STATE OF THE ART: We searched PubMed with keyword 'central liponeurocytoma' and the references of the related articles. There were no language or year restrictions. We included articles focusing on liponeurocytomas located in the central nervous system leaving a total of 17 articles and 21 reported cases. CLINICAL IMPLICATIONS: A 62-year-old female presented with confusion and mental disorientation without any other neurological deficit. Her magnetic resonance imaging (MRI) revealed a lateral ventricle located mass lesion which was hypointense on T1-weighted images (WI) and heterogeneously hyperintense on T2-WI with cystic component. Via craniotomy, yellow-beige colored, soft and moderately vascularized mass lesion was gross totally resected. Despite postoperative MRI revealed total resection, patient had left-sided hemiparesis. The patient recovered well in her postoperative period and there was no recurrence on her 6th month follow-up MRI. FUTURE DIRECTIONS: Intraventricular liponeurocytoma has a favorable clinical course, and radiological features may be useful in the diagnosis of this rare tumor before surgery. Supratentorial intraventricular location should be kept in mind in the differential diagnosis of the lateral ventricular tumors.
Asunto(s)
Neoplasias Cerebelosas , Lipoma , Neurocitoma , Diagnóstico Diferencial , Femenino , Humanos , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
OBJECTIVE: Liponeurocytoma is a rare benign tumor of the central nervous system, which develops mainly in adult patients within the posterior fossa. The World Health Organization has categorized this entity in its last classification of 2016 as a benign grade II tumor. Histopathologic characteristics contain neuronal and variable astrocytic differentiation with foci of lipomatous distinction. Only a few case reports and case series have been reported and the knowledge of this tumor is limited. General treatment guidelines do not exist. The aim of this study was to analyze the literature to create treatment guidelines. METHODS: PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines were followed to search existing online databases between January 1, 1978 and May 15, 2018. RESULTS: A systematic review of the literature found 73 patients (40 female, 33 male) with liponeurocytoma, in 59 of whom (80.8%) the disease occurred in the posterior fossa. The Ki-67/MIB-1 proliferation index was described in 58 patients, showing a mean value of 3.73% ± 4.01%. Follow-up was performed in 49 patients, with a median length of 52.02 ± 50.52 months, showing tumor recurrence in 14 patients (28.57%). Tumor recurrence was observed in only 1 patient (1/12, 8.33%) after adjuvant radiotherapy, whereas tumor recurrence was seen in 13/29 patients (44.83%) who did not receive adjuvant radiotherapy. CONCLUSIONS: Liponeurocytomas are rare benign tumors, occurring mainly in the cerebellum. The therapy of choice is surgery. Postoperative radiotherapy seems to decrease the risk of tumor recurrence and should be offered to the patient.
Asunto(s)
Neoplasias Cerebelosas/cirugía , Lipoma/cirugía , Neurocitoma/cirugía , Astrocitos/patología , Biopsia , Encéfalo/patología , Encéfalo/cirugía , Diferenciación Celular/fisiología , Neoplasias Cerebelosas/diagnóstico , Neoplasias Cerebelosas/patología , Terapia Combinada , Fosa Craneal Posterior/patología , Fosa Craneal Posterior/cirugía , Estudios de Seguimiento , Humanos , Lipoma/diagnóstico , Lipoma/patología , Recurrencia Local de Neoplasia/diagnóstico , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/cirugía , Neoplasia Residual/diagnóstico , Neoplasia Residual/patología , Neoplasia Residual/cirugía , Neurocitoma/diagnóstico , Neurocitoma/patología , Neuronas/patología , Guías de Práctica Clínica como Asunto , Radioterapia AdyuvanteRESUMEN
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.
Asunto(s)
Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/terapia , Neurocitoma/diagnóstico por imagen , Neurocitoma/terapia , Adulto , Neoplasias Cerebelosas/patología , Femenino , Humanos , Recurrencia Local de Neoplasia/diagnóstico por imagen , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/terapia , Neurocitoma/patologíaRESUMEN
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.
Asunto(s)
Neoplasias Cerebelosas/patología , Lipoma/patología , Neurocitoma/patología , Adulto , Neoplasias Cerebelosas/diagnóstico por imagen , Neoplasias Cerebelosas/cirugía , Humanos , Lipoma/diagnóstico por imagen , Lipoma/cirugía , Imagen por Resonancia Magnética , Masculino , Neurocitoma/diagnóstico por imagen , Neurocitoma/cirugíaRESUMEN
BACKGROUND: Liponeurocytoma is a very rare tumor classified as grade II (neuronal and mixed neuronal-glial tumors) according to 2016 World Health Organization classification of tumors of the central nervous system. The median age at detection is 50 years, and the most frequent location is the posterior cranial fossa, especially within the cerebellar hemispheres; liponeurocytomas arising in the cerebellopontine angle (CPA) are exceptional. CASE DESCRIPTION: Here we report the clinical, radiological, and pathological characteristics of a CPA liponeurocytoma in a 35-year-old woman, as well as a review of the literature. This unusual cisternal location raises the issue of the differential imaging diagnosis with much more common CPA tumors (e.g., meningiomas, vestibular schwannomas, ependymomas, epidermoid cyst, hemangioblastomas, medulloblastomas). CONCLUSION: To the best of our knowledge, 59 cases of cerebellar liponeurocytomas have been reported to date, which include only 6 cases of CPA liponeurocytomas. Treatment relies on total removal whenever possible, with an excellent prognosis, but a high MIB-1 index (>10%) and/or incomplete tumor resection are the main adverse prognostic factors.
Asunto(s)
Neoplasias Encefálicas/patología , Ángulo Pontocerebeloso/patología , Lipoma/patología , Neurocitoma/patología , Adulto , Diagnóstico Diferencial , Femenino , HumanosRESUMEN
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.