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Adult hemispheric cerebellar medulloblastoma

BACKGROUND: Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. It is associated with many familiar cancer syndromes, but there is no known cause for medulloblastoma. Many studies have documented differences between childhood and adult medulloblastomas in t...

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Autores principales: de Oliveira, Felipe, Landeiro, José Alberto, de Castro, Igor
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5838831/
https://www.ncbi.nlm.nih.gov/pubmed/29527392
http://dx.doi.org/10.4103/sni.sni_341_17
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author de Oliveira, Felipe
Landeiro, José Alberto
de Castro, Igor
author_facet de Oliveira, Felipe
Landeiro, José Alberto
de Castro, Igor
author_sort de Oliveira, Felipe
collection PubMed
description BACKGROUND: Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. It is associated with many familiar cancer syndromes, but there is no known cause for medulloblastoma. Many studies have documented differences between childhood and adult medulloblastomas in terms of location, proliferation, and apoptotic indices. There are four histological groups – classic and the variant forms (desmoplastic/nodular, anaplasic, and large cell). There are four major subgroups according to molecular configuration: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4 with differences between them according to prognostic outcomes. CASE DESCRIPTION: We present the case of a 19-year-old female who complained of headache and vomiting. On neurological exam, she was awake, conscious, and had mild truncal ataxia, dysmetria, and intentional tremor. Brain magnetic resonance imaging (MRI) showed an intra-axial left hemisphere cerebellar lesion causing midline shift tonsilar herniation. She was submitted for posterior fossa craniotomy and microsurgical resection of cerebellar tumor and then to 18 Gy adjuvant radiotherapy to the tumor bed and 23 Gy to the neuroaxis. CONCLUSION: This article briefly discusses the newest points in classification, diagnosis, and treatment of medulloblastoma. This case illustrates the diagnostic workup and treatment of a rare tumor in adults showing the importance of molecular and histological studies for the treatment and counseling of the patient. Medulloblastoma has different prognosis depending on the histological and molecular feature. Accessing these different features is essential to better plan the treatment as well as inform the patient regarding the disease and its prognosis.
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spelling pubmed-58388312018-03-09 Adult hemispheric cerebellar medulloblastoma de Oliveira, Felipe Landeiro, José Alberto de Castro, Igor Surg Neurol Int Neuroanatomy and Neurophysiology: Case Report BACKGROUND: Medulloblastoma is an embryonal neoplasm and accounts for 1% of all adult intracranial tumors. It is associated with many familiar cancer syndromes, but there is no known cause for medulloblastoma. Many studies have documented differences between childhood and adult medulloblastomas in terms of location, proliferation, and apoptotic indices. There are four histological groups – classic and the variant forms (desmoplastic/nodular, anaplasic, and large cell). There are four major subgroups according to molecular configuration: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4 with differences between them according to prognostic outcomes. CASE DESCRIPTION: We present the case of a 19-year-old female who complained of headache and vomiting. On neurological exam, she was awake, conscious, and had mild truncal ataxia, dysmetria, and intentional tremor. Brain magnetic resonance imaging (MRI) showed an intra-axial left hemisphere cerebellar lesion causing midline shift tonsilar herniation. She was submitted for posterior fossa craniotomy and microsurgical resection of cerebellar tumor and then to 18 Gy adjuvant radiotherapy to the tumor bed and 23 Gy to the neuroaxis. CONCLUSION: This article briefly discusses the newest points in classification, diagnosis, and treatment of medulloblastoma. This case illustrates the diagnostic workup and treatment of a rare tumor in adults showing the importance of molecular and histological studies for the treatment and counseling of the patient. Medulloblastoma has different prognosis depending on the histological and molecular feature. Accessing these different features is essential to better plan the treatment as well as inform the patient regarding the disease and its prognosis. Medknow Publications & Media Pvt Ltd 2018-02-14 /pmc/articles/PMC5838831/ /pubmed/29527392 http://dx.doi.org/10.4103/sni.sni_341_17 Text en Copyright: © 2018 Surgical Neurology International http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
spellingShingle Neuroanatomy and Neurophysiology: Case Report
de Oliveira, Felipe
Landeiro, José Alberto
de Castro, Igor
Adult hemispheric cerebellar medulloblastoma
title Adult hemispheric cerebellar medulloblastoma
title_full Adult hemispheric cerebellar medulloblastoma
title_fullStr Adult hemispheric cerebellar medulloblastoma
title_full_unstemmed Adult hemispheric cerebellar medulloblastoma
title_short Adult hemispheric cerebellar medulloblastoma
title_sort adult hemispheric cerebellar medulloblastoma
topic Neuroanatomy and Neurophysiology: Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5838831/
https://www.ncbi.nlm.nih.gov/pubmed/29527392
http://dx.doi.org/10.4103/sni.sni_341_17
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