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Primary germinoma of the medulla oblongata: illustrative case

BACKGROUND: Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South America...

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Autores principales: Albiña, Pablo, Solis, Aracelly, Lorenzoni, Jose, Henny, Pablo, Manriquez, María
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association of Neurological Surgeons 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9204933/
https://www.ncbi.nlm.nih.gov/pubmed/35733824
http://dx.doi.org/10.3171/CASE21315
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author Albiña, Pablo
Solis, Aracelly
Lorenzoni, Jose
Henny, Pablo
Manriquez, María
author_facet Albiña, Pablo
Solis, Aracelly
Lorenzoni, Jose
Henny, Pablo
Manriquez, María
author_sort Albiña, Pablo
collection PubMed
description BACKGROUND: Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South American case published to date, to the authors’ knowledge. OBSERVATIONS: Initially, the radiological differential diagnosis did not include this entity. The lesion was located at the obex and exhibited a well-delineated contrast enhancement without hydrocephalus. An emergency decompressive partial resection following functional limits was performed. After histological confirmation, radiotherapy was indicated, with complete remission achieved at a 6-month follow-up. The patient, however, continued to have a severe proprioceptive disorder. The literature review identified 21 other such patients. The mean age for this location was 23 years, with a strong female and Asian origin predilection. All tumors exhibited contrast enhancement, and only one presented with hydrocephalus. LESSONS: In the absence of elevated tumor markers, radiological clues such as a well-delineated, contrast-enhanced lesion arising from the obex, without hydrocephalus, associated with demographic features such as young age, female sex, and Asian heritage, should evoke a high level of suspicion for this diagnosis. Gross total resection must not be attempted, because this tumor is potentially curable with high-dose radiotherapy.
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spelling pubmed-92049332022-06-21 Primary germinoma of the medulla oblongata: illustrative case Albiña, Pablo Solis, Aracelly Lorenzoni, Jose Henny, Pablo Manriquez, María J Neurosurg Case Lessons Case Lesson BACKGROUND: Primary central nervous system germinomas of the medulla oblongata are extremely rare and usually have been found in young female Asian patients. The authors present an illustrative case of a patient who presented with severe medullary and posterior cord syndrome, the first South American case published to date, to the authors’ knowledge. OBSERVATIONS: Initially, the radiological differential diagnosis did not include this entity. The lesion was located at the obex and exhibited a well-delineated contrast enhancement without hydrocephalus. An emergency decompressive partial resection following functional limits was performed. After histological confirmation, radiotherapy was indicated, with complete remission achieved at a 6-month follow-up. The patient, however, continued to have a severe proprioceptive disorder. The literature review identified 21 other such patients. The mean age for this location was 23 years, with a strong female and Asian origin predilection. All tumors exhibited contrast enhancement, and only one presented with hydrocephalus. LESSONS: In the absence of elevated tumor markers, radiological clues such as a well-delineated, contrast-enhanced lesion arising from the obex, without hydrocephalus, associated with demographic features such as young age, female sex, and Asian heritage, should evoke a high level of suspicion for this diagnosis. Gross total resection must not be attempted, because this tumor is potentially curable with high-dose radiotherapy. American Association of Neurological Surgeons 2022-06-06 /pmc/articles/PMC9204933/ /pubmed/35733824 http://dx.doi.org/10.3171/CASE21315 Text en © 2022 The authors https://creativecommons.org/licenses/by-nc-nd/4.0/CC BY-NC-ND 4.0 (http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) ).
spellingShingle Case Lesson
Albiña, Pablo
Solis, Aracelly
Lorenzoni, Jose
Henny, Pablo
Manriquez, María
Primary germinoma of the medulla oblongata: illustrative case
title Primary germinoma of the medulla oblongata: illustrative case
title_full Primary germinoma of the medulla oblongata: illustrative case
title_fullStr Primary germinoma of the medulla oblongata: illustrative case
title_full_unstemmed Primary germinoma of the medulla oblongata: illustrative case
title_short Primary germinoma of the medulla oblongata: illustrative case
title_sort primary germinoma of the medulla oblongata: illustrative case
topic Case Lesson
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9204933/
https://www.ncbi.nlm.nih.gov/pubmed/35733824
http://dx.doi.org/10.3171/CASE21315
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