Cerebellar Liponeurocytoma: Relevant Clinical Cytogenetic Findings

Tucker, Alexander M.; Boon-Unge, Kritsanapol; McLaughlin, Nancy; Ibrahim, Hassana; Rao, Nagesh; Martin, Neil A.; Everson, Richard; Khanlou, Négar

doi:10.4132/jptm.2016.07.24

Cited by 10 publications

(11 citation statements)

References 10 publications

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“…Immunostaining provides additional meaningful information that allows for correct diagnosis and differential diagnosis. cLNCs immunohistochemical analyses were positive for SYN, GFAP, NSE, and MAP-2, and negative for IDH-1 mutations, oligo-2, and EMA ( 10 , 11 , 23 , 31 ). In addition, enhanced levels of the adipocyte-associated transcription factor, NEUROG1, and fatty acid-binding protein 4, have been described in cLNCs ( 14 ).…”

Section: Discussionmentioning

confidence: 97%

“…Notably, mild to moderate peritumoral edema was observed in three pediatric patients (3/4), with two cases of mild, and one case of moderate surrounding edema. However, peritumoral edema was observed only in 9 adult patients (9/63, [14.3%]) ( 16 , 23 – 30 ), as was mild edema. In some studies, the metabolic activity of cLNCs was evaluated with positron emission tomography (PET) ( 7 , 31 ).…”

Section: Discussionmentioning

confidence: 99%

“…This was also true for the case reported by Nzegwu et al who also only received GTR ( 13 ). Regarding patients with incomplete tumor resection (ITR) and/or a high Ki-67 index, postoperative radiotherapy is strongly recommended ( 23 ). Jouvet et al reported a cLNC with only ITR (Ki-67 index of 10-15%), and the tumor relapsed 14-month later (Ki-67 index of 15-30%) ( 12 ).…”

Section: Discussionmentioning

confidence: 99%

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Cerebellar Liponeurocytoma Mimicking Medulloblastoma: Case Report of a Childhood and Literature Review

et al. 2021

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BackgroundCerebellar 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 DescriptionA 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.ConclusionThe 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.

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Cerebellar Liponeurocytoma Mimicking Medulloblastoma: Case Report of a Childhood and Literature Review

et al. 2021

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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 ].…”

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 deletion of isochromosome 17, combined with the absence of mutations in the adenomatous polyposis coli gene ( APC ), the protein patched homolog gene ( PTCH ), and the β‐catenin gene ( CTNNB ), supported that CL represents a distinct entity, molecularly different from both MB and neurocytoma 6 . Recently, it has been demonstrated that CL exhibited the neurogenin‐1 gene ( NEUROG1 ) transcripts, presenting non‐pathological conditions in cerebellar progenitor cells of the ventricular zone, and lacked transcripts of the atonal homolog 1 gene ( ATOH1 ) and the orthodenticle homeobox 2 gene ( OTX2 ), both found in progenitor cells of the external granule cell layer 14 . Based on these data, it has been hypothesized that CL may arise from γ‐aminobutyric acid (GABA)ergic neurons of the cerebellar ventricular zone 14 …”

Section: Introductionmentioning

confidence: 92%

Cerebellar liponeurocytoma: clinical, histopathological and molecular features of a series of three cases, including one recurrent tumor

et al. 2022

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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.

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Cerebellar Liponeurocytoma: Relevant Clinical Cytogenetic Findings

Cited by 10 publications

References 10 publications

Cerebellar Liponeurocytoma Mimicking Medulloblastoma: Case Report of a Childhood and Literature Review

Cerebellar Liponeurocytoma Mimicking Medulloblastoma: Case Report of a Childhood and Literature Review

Cerebellar liponeurocytoma – a rare entity: a case report

Cerebellar liponeurocytoma: clinical, histopathological and molecular features of a series of three cases, including one recurrent tumor

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