The evolution of the anaplastic cerebellar liponeurocytoma: case report and review of the literature

Radke, Josefine; Gehlhaar, Claire; Lenze, Dido; Capper, David; Bock, Alexander; Heppner, Frank L.; Jödicke, Andreas; Koch, Arend

doi:10.5414/np300783

Cited by 17 publications

(14 citation statements)

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“…The Ki 67/MIB-1 index is usually low (< 5%); however, patients with a relatively high proliferation rate and cytopathologic atypia have been re- ported to have a greater risk of recurrence and, therefore, a poorer prognosis. 2,7,14,20 There is currently no established standard of care, but maximal safe resection followed by postoperative imaging surveillance for recurrence seems prudent. The roles of adjuvant chemotherapy and radiation have not been established.…”

Section: Discussionmentioning

confidence: 99%

Cerebellar liponeurocytoma: a rare intracranial tumor with possible familial predisposition. Case report

Wolf

Alghefari²,

Krivosheya

et al. 2016

JNS

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C erebellar liponeurocytomas are typically lowgrade tumors that arise in the cerebellar hemispheres or vermis and contain cells of neuronal, astrocytic, and lipomatous differentiation. 17 The biology and long-term outcomes of these tumors are undefined, and to our knowledge, familial occurrences have not been described. We present here the case of a young woman with a cerebellar liponeurocytoma and multiple immediate family members, including her mother, with similar lesions. This report describes the tumors from the patient and her mother and provides a brief overview of the histopathology and genetic changes associated with this disease. case report Clinical PresentationA 37-year-old woman presented with a 2-year history of worsening morning and daytime headaches in the occipital area that radiated over the cranium. She also described intermittent paresthesia in her hands and feet, which was of variable distribution and duration. The patient was otherwise healthy with a grossly normal neurological examination. She smoked cigarettes but had no other known medical problems. CT and MRI of the head and spine revealed a solitary fat-containing tumor arising from the region of the superior cerebellar hemisphere and vermis. The heterogeneous lesion had numerous ill-defined septations with patchy enhancement and measured 4.4 × 4.1 × 4.3 cm in the anterior-posterior, medial-lateral, and superior-inferior dimensions, respectively. It extended through the incisura and involved the tectal plate and several deep veins, including the left basal vein of Rosenthal. There was complete effacement of the fourth ventricle and compensated dilation of the third and lateral ventricles. There was displacement of the cerebellar tonsils through the foramen magnum, with compression of the cervicomedullary junction ( Fig. 1A and B).abbreviatioNs GFAP = glial fibrillary acidic protein; IDH1 = isocitrate dehydrogenase 1; MAP-2 = microtubule-associated protein 2; MIB-1 = monoclonal antihuman Ki 67. 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 previo...

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Section: Discussionmentioning

confidence: 99%

Cerebellar liponeurocytoma: a rare intracranial tumor with possible familial predisposition. Case report

Wolf

Alghefari²,

Krivosheya

et al. 2016

JNS

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“…In a study, tumor recurrence was observed in 50% of the patients with cerebellar liponeurocytoma who had not received radiotherapy following tumor surgery, but no tumor recurrence was reported in patients undergoing radiotherapy after surgery [23]. According to various studies, high Ki-67 positive cells (more than 10%) and subtotal tumor resection of the tumor are two main risk factors for tumor recurrence [6,23].…”

Section: Discussionmentioning

confidence: 99%

Liponeurocytoma of the Cerebellopontine Angle: An Interesting Case Report and Literature Review

et al. 2020

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Background and Importance: Liponeurocytoma is a rare tumor of the CNS. Some of its prominent features include low neuronal proliferation with foci of lipomatous differentiation. So far, 70 reports on cases of brain liponeurocytoma have been published. Most of them were located in the cerebellar hemisphere, but only 7 cases of primary Cerebellopontine Angle (CPA) liponeurocytoma have been reported. Case Presentation: We presented a 27-year-old woman with a 6-month progressive headache and dizziness. More recently, she developed progressive hearing loss and ataxia. Pre-operative MRI of the brain demonstrated a heterogeneous solid extra-axial lesion in the right CPA with hydrocephalus. The operation was performed via the retro sigmoid approach. Histopathological diagnosis was confirmed as liponeurocytoma. No radiotherapy was performed. The patient was followed up for two years. There were no signs of recurrence. Conclusion: Liponeurocytoma is a rare tumor. In most of the cases, it is presented in the cerebellum, and the CPA as the primary location of this tumor is extremely uncommon. Typically, surgery is the treatment of choice for these lesions. If the tumor is subtotal, radiotherapy after operation and resection can also be advantageous.

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“…Fluorescent in situ hybridization studies of a liponeurocytoma showed no evidence of 1p/19q co-deletion, N-MYC or epidermal growth factor receptor amplification, MYC or EWSR1 rearrangements, or PTEN deletion [ 22 , 23 ]. In contrast to these findings, large cell/anaplastic medulloblastomas are associated with C-MYC or N-MYC amplifications [ 23 ]. Despite this, Wolf et al [ 24 ] and Pikis et al .…”

Section: Discussionmentioning

confidence: 99%

“…Radke et al . were able to show an increase of Ki-67 positive cells in recurrent tumors [ 23 ]. Due to the potentially long-term malignancy, close follow-up is recommended over that period [ 27 , 28 ].…”

Section: Discussionmentioning

confidence: 99%

Cerebellar liponeurocytoma – a rare entity: a case report

et al. 2018

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BackgroundCerebellar 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 presentationA 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.ConclusionsLiponeurocytomas 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.

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The evolution of the anaplastic cerebellar liponeurocytoma: case report and review of the literature

Cited by 17 publications

References 0 publications

Cerebellar liponeurocytoma: a rare intracranial tumor with possible familial predisposition. Case report

Cerebellar liponeurocytoma: a rare intracranial tumor with possible familial predisposition. Case report

Liponeurocytoma of the Cerebellopontine Angle: An Interesting Case Report and Literature Review

Cerebellar liponeurocytoma – a rare entity: a case report

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