An advanced well-differentiated pancreatic neuroendocrine carcinoma (NET-G3) associated with von hippel-lindau disease

Masami Miki, Ken Kawabe, Hisato Igarashi, Tatsuro Abe, Yoshihiro Ohishi, Risa Hashimoto, Takashi Karashima, Ichiro Yamasaki, Keiji Inoue, Tetsuhide Ito, Yoshihiro Ogawa

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1 Citation (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)2007-2011
Number of pages5
JournalInternal Medicine
Volume57
Issue number14
DOIs
Publication statusPublished - 2018

All Science Journal Classification (ASJC) codes

  • Internal Medicine

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