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Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review
Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cor...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Codon Publications
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10219837/ https://www.ncbi.nlm.nih.gov/pubmed/37251595 http://dx.doi.org/10.15586/jkcvhl.v10i2.272 |
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author | Coco, Danilo Leanza, Silvana |
author_facet | Coco, Danilo Leanza, Silvana |
author_sort | Coco, Danilo |
collection | PubMed |
description | Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cord, renal clear cell carcinoma (RCCC), paraganglioma, and neuroendocrine tumors. There may also be lymphangiomas, epididymal cysts, and pancreatic cysts or pancreatic neuroendocrine tumors (pNETs). The most frequent causes of death are metastasis from RCCC and neurological complications from retinoblastoma or central nervous system (CNS). Pancreatic cysts are present in 35–70% of VHL patients. Simple cysts, serous cysts, or pNETs are possible presentations, and the likelihood of malignant degeneration or metastasis is no greater than 8%. Although VHL has been associated with pNETs, their pathological characteristics are unknown. Furthermore, it is unknown whether variations in the VHL gene cause the development of pNETs. Hence, this retrospective study was undertaken with the main aim to examine whether pNETs are connected to VHL from a surgical perspective. |
format | Online Article Text |
id | pubmed-10219837 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Codon Publications |
record_format | MEDLINE/PubMed |
spelling | pubmed-102198372023-05-28 Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review Coco, Danilo Leanza, Silvana J Kidney Cancer VHL Vhl and Vhl Related Disorders Multiorgan tumors are a hallmark of the autosomal dominant genetic disorder known as Von Hippel-Lindau syndrome (VHL), which is typically the result of inherited aberrations of the VHL tumor suppressor gene. The most frequent cancer is retinoblastoma, which can also occur in the brain and spinal cord, renal clear cell carcinoma (RCCC), paraganglioma, and neuroendocrine tumors. There may also be lymphangiomas, epididymal cysts, and pancreatic cysts or pancreatic neuroendocrine tumors (pNETs). The most frequent causes of death are metastasis from RCCC and neurological complications from retinoblastoma or central nervous system (CNS). Pancreatic cysts are present in 35–70% of VHL patients. Simple cysts, serous cysts, or pNETs are possible presentations, and the likelihood of malignant degeneration or metastasis is no greater than 8%. Although VHL has been associated with pNETs, their pathological characteristics are unknown. Furthermore, it is unknown whether variations in the VHL gene cause the development of pNETs. Hence, this retrospective study was undertaken with the main aim to examine whether pNETs are connected to VHL from a surgical perspective. Codon Publications 2023-05-23 /pmc/articles/PMC10219837/ /pubmed/37251595 http://dx.doi.org/10.15586/jkcvhl.v10i2.272 Text en Copyright: Coco D and Leanza S https://creativecommons.org/licenses/by/4.0/License: This open access article is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0). http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/) |
spellingShingle | Vhl and Vhl Related Disorders Coco, Danilo Leanza, Silvana Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title | Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title_full | Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title_fullStr | Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title_full_unstemmed | Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title_short | Von Hippel-Lindau is Associated to Pancreatic Neuroendocrine Tumors: A Comprehensive Review |
title_sort | von hippel-lindau is associated to pancreatic neuroendocrine tumors: a comprehensive review |
topic | Vhl and Vhl Related Disorders |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10219837/ https://www.ncbi.nlm.nih.gov/pubmed/37251595 http://dx.doi.org/10.15586/jkcvhl.v10i2.272 |
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