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Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease
Hemangioblastoma (HB) of the central nervous system may occur sporadically or in association with von Hippel-Lindau (VHL) disease. Disseminated HB means malignant spread of the original primary HB without local recurrence at surgically resected site. It has been rarely reported previously, and rarer...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Korean Brain Tumor Society; The Korean Society for Neuro-Oncology
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4231619/ https://www.ncbi.nlm.nih.gov/pubmed/25408933 http://dx.doi.org/10.14791/btrt.2014.2.2.96 |
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author | Chung, Sun-Yoon Jeun, Sin-Soo Park, Jae-Hyun |
author_facet | Chung, Sun-Yoon Jeun, Sin-Soo Park, Jae-Hyun |
author_sort | Chung, Sun-Yoon |
collection | PubMed |
description | Hemangioblastoma (HB) of the central nervous system may occur sporadically or in association with von Hippel-Lindau (VHL) disease. Disseminated HB means malignant spread of the original primary HB without local recurrence at surgically resected site. It has been rarely reported previously, and rarer especially without VHL gene mutation. We report a case of disseminated HB without VHL disease. A 59-year-old man underwent a surgery for total removal of a cerebellar HB. From five years after the surgery, multiple dissemination of HB was identified intracranially and he subsequently underwent cyberknife radiosurgery. The lesions got smaller temporarily, but they soon grew larger. Nine years after the initial surgery for cerebellar HB, he showed severe back pain. His magnetic resonance image of spine revealed intradural extramedullary mass at T6-7 level. Complete surgical removal of the mass was performed and the pathological diagnosis was identical to the previous one. He had no evidence of VHL disease. And there was no recurrence of the tumor at the site of the original operation. The exact mechanism of dissemination is unknown, but the surgeon should be cautious of tumor cell spillage during surgery and prudently consider the decision to perform ventriculo-peritoneal shunt. In addition, continuous follow-up for recurrence or dissemination is necessary for patients even who underwent complete removal of cerebellar HB. |
format | Online Article Text |
id | pubmed-4231619 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | The Korean Brain Tumor Society; The Korean Society for Neuro-Oncology |
record_format | MEDLINE/PubMed |
spelling | pubmed-42316192014-11-18 Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease Chung, Sun-Yoon Jeun, Sin-Soo Park, Jae-Hyun Brain Tumor Res Treat Case Report Hemangioblastoma (HB) of the central nervous system may occur sporadically or in association with von Hippel-Lindau (VHL) disease. Disseminated HB means malignant spread of the original primary HB without local recurrence at surgically resected site. It has been rarely reported previously, and rarer especially without VHL gene mutation. We report a case of disseminated HB without VHL disease. A 59-year-old man underwent a surgery for total removal of a cerebellar HB. From five years after the surgery, multiple dissemination of HB was identified intracranially and he subsequently underwent cyberknife radiosurgery. The lesions got smaller temporarily, but they soon grew larger. Nine years after the initial surgery for cerebellar HB, he showed severe back pain. His magnetic resonance image of spine revealed intradural extramedullary mass at T6-7 level. Complete surgical removal of the mass was performed and the pathological diagnosis was identical to the previous one. He had no evidence of VHL disease. And there was no recurrence of the tumor at the site of the original operation. The exact mechanism of dissemination is unknown, but the surgeon should be cautious of tumor cell spillage during surgery and prudently consider the decision to perform ventriculo-peritoneal shunt. In addition, continuous follow-up for recurrence or dissemination is necessary for patients even who underwent complete removal of cerebellar HB. The Korean Brain Tumor Society; The Korean Society for Neuro-Oncology 2014-10 2014-10-31 /pmc/articles/PMC4231619/ /pubmed/25408933 http://dx.doi.org/10.14791/btrt.2014.2.2.96 Text en Copyright © 2014 The Korean Brain Tumor Society and The Korean Society for Neuro-Oncology http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Chung, Sun-Yoon Jeun, Sin-Soo Park, Jae-Hyun Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title | Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title_full | Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title_fullStr | Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title_full_unstemmed | Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title_short | Disseminated Hemangioblastoma of the Central Nervous System without Von Hippel-Lindau Disease |
title_sort | disseminated hemangioblastoma of the central nervous system without von hippel-lindau disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4231619/ https://www.ncbi.nlm.nih.gov/pubmed/25408933 http://dx.doi.org/10.14791/btrt.2014.2.2.96 |
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