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Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports

RATIONALE: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features. PATIENT CONCERNS: In this article, we report two cases of intracranial medulloepitheliomas. In the firs...

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Autores principales: Li, Qiang, Chen, Ni, Ju, Yan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5976342/
https://www.ncbi.nlm.nih.gov/pubmed/29768355
http://dx.doi.org/10.1097/MD.0000000000010751
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author Li, Qiang
Chen, Ni
Ju, Yan
author_facet Li, Qiang
Chen, Ni
Ju, Yan
author_sort Li, Qiang
collection PubMed
description RATIONALE: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features. PATIENT CONCERNS: In this article, we report two cases of intracranial medulloepitheliomas. In the first patient, a one-month old boy, the tumor was located in in right lateral ventricle, which was the first report of such location of this disease; in the second patient, an eleven-month old girl, the tumor was in right cerebellopontine angle. DIAGNOSES: Both patients were diagnosed as medulloepithelioma by pathologists. INTERVENTIONS: Both patients underwent craniotomy to resect the lesion totally. OUTCOMES: The boy underwent chemotherapy after operation and was alive 3 months after operation. The girl died 6 months after operation, despite aggressive adjuvant chemotherapy. LESSONS: Surgical resection is safe and effective to prolong patient survival. However, despite aggressive adjuvant therapy, prognosis of medulloepithelioma remains poor, and further study is needed to improve treatment of this rare disease.
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spelling pubmed-59763422018-06-05 Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports Li, Qiang Chen, Ni Ju, Yan Medicine (Baltimore) Research Article RATIONALE: Intracranial medulloepitheliomas are extremely rare and highly malignant. Intraventricular medulloepitheliomas are even rarely reported, and little is known about the clinical features. PATIENT CONCERNS: In this article, we report two cases of intracranial medulloepitheliomas. In the first patient, a one-month old boy, the tumor was located in in right lateral ventricle, which was the first report of such location of this disease; in the second patient, an eleven-month old girl, the tumor was in right cerebellopontine angle. DIAGNOSES: Both patients were diagnosed as medulloepithelioma by pathologists. INTERVENTIONS: Both patients underwent craniotomy to resect the lesion totally. OUTCOMES: The boy underwent chemotherapy after operation and was alive 3 months after operation. The girl died 6 months after operation, despite aggressive adjuvant chemotherapy. LESSONS: Surgical resection is safe and effective to prolong patient survival. However, despite aggressive adjuvant therapy, prognosis of medulloepithelioma remains poor, and further study is needed to improve treatment of this rare disease. Wolters Kluwer Health 2018-05-18 /pmc/articles/PMC5976342/ /pubmed/29768355 http://dx.doi.org/10.1097/MD.0000000000010751 Text en Copyright © 2018 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nd/4.0 This is an open access article distributed under the Creative Commons Attribution-NoDerivatives License 4.0, which allows for redistribution, commercial and non-commercial, as long as it is passed along unchanged and in whole, with credit to the author. http://creativecommons.org/licenses/by-nd/4.0
spellingShingle Research Article
Li, Qiang
Chen, Ni
Ju, Yan
Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title_full Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title_fullStr Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title_full_unstemmed Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title_short Infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: Two case reports
title_sort infantile medulloepithelioma in the lateral ventricle and cerebellopontine angle: two case reports
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5976342/
https://www.ncbi.nlm.nih.gov/pubmed/29768355
http://dx.doi.org/10.1097/MD.0000000000010751
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AT chenni infantilemedulloepitheliomainthelateralventricleandcerebellopontineangletwocasereports
AT juyan infantilemedulloepitheliomainthelateralventricleandcerebellopontineangletwocasereports