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Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature

Medulloblastoma is an embryonal neuroepithelial tumor that affects mainly childhood and more rarely adults. Medulloblastoma occurring as multiple nodules at diagnosis is a rare and tricky presentation. Here, we describe the case of a previously healthy 47-year-old woman with multiple posterior fossa...

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Autores principales: Troncon, Irene, Guerriero, Angela, Rossi, Sabrina, Ronzon, Monica, Padovan, Marta, Mario, Caccese, Zanatta, Lucia, Toffolatti, Luisa, Marton, Elisabetta, Lombardi, Giuseppe, Dei Tos, Angelo Paolo, Canova, Giuseppe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7503105/
https://www.ncbi.nlm.nih.gov/pubmed/33005468
http://dx.doi.org/10.1155/2020/4502878
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author Troncon, Irene
Guerriero, Angela
Rossi, Sabrina
Ronzon, Monica
Padovan, Marta
Mario, Caccese
Zanatta, Lucia
Toffolatti, Luisa
Marton, Elisabetta
Lombardi, Giuseppe
Dei Tos, Angelo Paolo
Canova, Giuseppe
author_facet Troncon, Irene
Guerriero, Angela
Rossi, Sabrina
Ronzon, Monica
Padovan, Marta
Mario, Caccese
Zanatta, Lucia
Toffolatti, Luisa
Marton, Elisabetta
Lombardi, Giuseppe
Dei Tos, Angelo Paolo
Canova, Giuseppe
author_sort Troncon, Irene
collection PubMed
description Medulloblastoma is an embryonal neuroepithelial tumor that affects mainly childhood and more rarely adults. Medulloblastoma occurring as multiple nodules at diagnosis is a rare and tricky presentation. Here, we describe the case of a previously healthy 47-year-old woman with multiple posterior fossa cerebellar tumors. Histological, immunohistochemical, and molecular analyses were performed to best characterize the two excised lesions. The histopathological analysis revealed different variants of medulloblastoma in the excised nodules, one being extensive nodularity, rare in adults, and the other desmoplastic/nodular with areas of anaplasia. Immunostains and molecular analysis classified both nodules as SHH medulloblastoma. Adult medulloblastoma is extremely rare. Important differences exist between adult medulloblastoma and medulloblastoma arising in children and infants. Such differences are in location, distribution of histological variants and of molecular subgroups, survival rates, and therapeutic options. An extensive morphological and molecular characterization of such rare tumors is necessary to choice the best-tailored therapy.
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spelling pubmed-75031052020-09-30 Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature Troncon, Irene Guerriero, Angela Rossi, Sabrina Ronzon, Monica Padovan, Marta Mario, Caccese Zanatta, Lucia Toffolatti, Luisa Marton, Elisabetta Lombardi, Giuseppe Dei Tos, Angelo Paolo Canova, Giuseppe Case Rep Pathol Case Report Medulloblastoma is an embryonal neuroepithelial tumor that affects mainly childhood and more rarely adults. Medulloblastoma occurring as multiple nodules at diagnosis is a rare and tricky presentation. Here, we describe the case of a previously healthy 47-year-old woman with multiple posterior fossa cerebellar tumors. Histological, immunohistochemical, and molecular analyses were performed to best characterize the two excised lesions. The histopathological analysis revealed different variants of medulloblastoma in the excised nodules, one being extensive nodularity, rare in adults, and the other desmoplastic/nodular with areas of anaplasia. Immunostains and molecular analysis classified both nodules as SHH medulloblastoma. Adult medulloblastoma is extremely rare. Important differences exist between adult medulloblastoma and medulloblastoma arising in children and infants. Such differences are in location, distribution of histological variants and of molecular subgroups, survival rates, and therapeutic options. An extensive morphological and molecular characterization of such rare tumors is necessary to choice the best-tailored therapy. Hindawi 2020-09-12 /pmc/articles/PMC7503105/ /pubmed/33005468 http://dx.doi.org/10.1155/2020/4502878 Text en Copyright © 2020 Irene Troncon et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Troncon, Irene
Guerriero, Angela
Rossi, Sabrina
Ronzon, Monica
Padovan, Marta
Mario, Caccese
Zanatta, Lucia
Toffolatti, Luisa
Marton, Elisabetta
Lombardi, Giuseppe
Dei Tos, Angelo Paolo
Canova, Giuseppe
Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title_full Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title_fullStr Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title_full_unstemmed Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title_short Multifocal Medulloblastoma in an Adult Patient: Description of a Rare Presentation and Review of the Literature
title_sort multifocal medulloblastoma in an adult patient: description of a rare presentation and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7503105/
https://www.ncbi.nlm.nih.gov/pubmed/33005468
http://dx.doi.org/10.1155/2020/4502878
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