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Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt
Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder characterized by multiple benign and malignant tumors involving different organs (renal, adrenal, pancreas, liver, urogenital system, central nervous system, and head and neck region) due to mutations in the VHL tumor suppressor gene....
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10613454/ https://www.ncbi.nlm.nih.gov/pubmed/37905285 http://dx.doi.org/10.7759/cureus.46205 |
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author | Santos, Mariana Marussi, Victor H Campos, Christiane M Doria-Netto, Hugo Leonardo Doria-Netto, Ricardo Henrique Chaddad-Neto, Feres Amaral, Lázaro Luís F |
author_facet | Santos, Mariana Marussi, Victor H Campos, Christiane M Doria-Netto, Hugo Leonardo Doria-Netto, Ricardo Henrique Chaddad-Neto, Feres Amaral, Lázaro Luís F |
author_sort | Santos, Mariana |
collection | PubMed |
description | Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder characterized by multiple benign and malignant tumors involving different organs (renal, adrenal, pancreas, liver, urogenital system, central nervous system, and head and neck region) due to mutations in the VHL tumor suppressor gene. Here, we describe a patient with unknown VHL disease who has complained of hypoesthesia of the right lower limb for about six years. A lumbar MRI was performed and revealed an expansive foraminal lesion at the right L3-L4 level and multiple serpiginous intradural and extramedullary flow voids involving the dorsal aspect of the spinal cord. The patient underwent digital subtraction angiography to exclude a spinal dural arteriovenous fistula, which revealed imaging features suggestive of spinal hemangioblastoma. In the presence of a spinal hemangioblastoma, a brain MRI was performed for further evaluation to rule out the possible diagnosis of VHL disease, and a solitary hemangioblastoma on the right cerebellar hemisphere was found. The patient underwent lumbar spine surgery, confirming the suspicious diagnosis of hemangioblastomas related to VHL disease. |
format | Online Article Text |
id | pubmed-10613454 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
spelling | pubmed-106134542023-10-30 Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt Santos, Mariana Marussi, Victor H Campos, Christiane M Doria-Netto, Hugo Leonardo Doria-Netto, Ricardo Henrique Chaddad-Neto, Feres Amaral, Lázaro Luís F Cureus Neurology Von Hippel-Lindau (VHL) disease is a rare neurocutaneous disorder characterized by multiple benign and malignant tumors involving different organs (renal, adrenal, pancreas, liver, urogenital system, central nervous system, and head and neck region) due to mutations in the VHL tumor suppressor gene. Here, we describe a patient with unknown VHL disease who has complained of hypoesthesia of the right lower limb for about six years. A lumbar MRI was performed and revealed an expansive foraminal lesion at the right L3-L4 level and multiple serpiginous intradural and extramedullary flow voids involving the dorsal aspect of the spinal cord. The patient underwent digital subtraction angiography to exclude a spinal dural arteriovenous fistula, which revealed imaging features suggestive of spinal hemangioblastoma. In the presence of a spinal hemangioblastoma, a brain MRI was performed for further evaluation to rule out the possible diagnosis of VHL disease, and a solitary hemangioblastoma on the right cerebellar hemisphere was found. The patient underwent lumbar spine surgery, confirming the suspicious diagnosis of hemangioblastomas related to VHL disease. Cureus 2023-09-29 /pmc/articles/PMC10613454/ /pubmed/37905285 http://dx.doi.org/10.7759/cureus.46205 Text en Copyright © 2023, Santos et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. |
spellingShingle | Neurology Santos, Mariana Marussi, Victor H Campos, Christiane M Doria-Netto, Hugo Leonardo Doria-Netto, Ricardo Henrique Chaddad-Neto, Feres Amaral, Lázaro Luís F Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title | Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title_full | Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title_fullStr | Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title_full_unstemmed | Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title_short | Unusual Spinal Foraminal Hemangioblastoma With Prominent Arteriovenous Shunt |
title_sort | unusual spinal foraminal hemangioblastoma with prominent arteriovenous shunt |
topic | Neurology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10613454/ https://www.ncbi.nlm.nih.gov/pubmed/37905285 http://dx.doi.org/10.7759/cureus.46205 |
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