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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Association of Neurological Surgeons
2022
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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. |
format | Online Article Text |
id | pubmed-9204933 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | American Association of Neurological Surgeons |
record_format | MEDLINE/PubMed |
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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