An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease

A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a...

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Autores principales: Miki, Masami, Kawabe, Ken, Igarashi, Hisato, Abe, Tatsuro, Ohishi, Yoshihiro, Hashimoto, Risa, Karashima, Takashi, Yamasaki, Ichiro, Inoue, Keiji, Ito, Tetsuhide, Ogawa, Yoshihiro
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2018
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6096011/
https://www.ncbi.nlm.nih.gov/pubmed/29491307
http://dx.doi.org/10.2169/internalmedicine.0416-17
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author Miki, Masami
Kawabe, Ken
Igarashi, Hisato
Abe, Tatsuro
Ohishi, Yoshihiro
Hashimoto, Risa
Karashima, Takashi
Yamasaki, Ichiro
Inoue, Keiji
Ito, Tetsuhide
Ogawa, Yoshihiro
author_facet Miki, Masami
Kawabe, Ken
Igarashi, Hisato
Abe, Tatsuro
Ohishi, Yoshihiro
Hashimoto, Risa
Karashima, Takashi
Yamasaki, Ichiro
Inoue, Keiji
Ito, Tetsuhide
Ogawa, Yoshihiro
author_sort Miki, Masami
collection PubMed
description A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease.
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spelling pubmed-60960112018-08-17 An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease Miki, Masami Kawabe, Ken Igarashi, Hisato Abe, Tatsuro Ohishi, Yoshihiro Hashimoto, Risa Karashima, Takashi Yamasaki, Ichiro Inoue, Keiji Ito, Tetsuhide Ogawa, Yoshihiro Intern Med Case Report A 45-year old woman who underwent several surgeries for tumors associated with von Hippel-Lindau disease (VHL) was referred to our hospital due to a pancreatic tumor and liver tumors. She was diagnosed with pancreatic neuroendocrine tumor (NET) with a Ki67 index of 40% based on the examination of a biopsy specimen of the liver tumors. She was treated with everolimus for 6 months and sunitinib for 6 weeks as first- and second-line therapies. She survived for 13 months. At autopsy the diagnosis of pancreatic neuroendocrine tumor (NET)-G3 was confirmed. We herein report an aggressive clinical course of VHL-related NET G3. The further accumulation of cases is required to reach a consensus on treatment for this disease. The Japanese Society of Internal Medicine 2018-02-28 2018-07-15 /pmc/articles/PMC6096011/ /pubmed/29491307 http://dx.doi.org/10.2169/internalmedicine.0416-17 Text en Copyright © 2018 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Miki, Masami
Kawabe, Ken
Igarashi, Hisato
Abe, Tatsuro
Ohishi, Yoshihiro
Hashimoto, Risa
Karashima, Takashi
Yamasaki, Ichiro
Inoue, Keiji
Ito, Tetsuhide
Ogawa, Yoshihiro
An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title_full An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title_fullStr An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title_full_unstemmed An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title_short An Advanced Well-differentiated Pancreatic Neuroendocrine Carcinoma (NET-G3) Associated with Von Hippel-Lindau Disease
title_sort advanced well-differentiated pancreatic neuroendocrine carcinoma (net-g3) associated with von hippel-lindau disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6096011/
https://www.ncbi.nlm.nih.gov/pubmed/29491307
http://dx.doi.org/10.2169/internalmedicine.0416-17
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