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

doi:10.2169/internalmedicine.0416-17

CASE REPORTS

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

Author information

Masami Miki
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan
Ken Kawabe
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan
Hisato Igarashi
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan
Tatsuro Abe
Department of Anatomic Pathology Science, Graduate School of Medical Sciences, Kyushu University, Japan
Yoshihiro Ohishi
Department of Anatomic Pathology Science, Graduate School of Medical Sciences, Kyushu University, Japan
Risa Hashimoto
Department of Gastroenterology, Saiseikai Fukuoka General Hospital, Japan
Takashi Karashima
Department of Urology, Kochi Medical School, Kochi University, Japan
Ichiro Yamasaki
Department of Urology, Kochi Medical School, Kochi University, Japan
Keiji Inoue
Department of Urology, Kochi Medical School, Kochi University, Japan
Tetsuhide Ito
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan
Yoshihiro Ogawa
Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Japan

Keywords: pancreatic neuroendocrine tumors-G3 (NET-G3), well-differentiated neuroendocrine carcinoma (WDNEC), von Hippel-Lindau disease (VHL), WHO G3, everolimus

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 14 Pages 2007-2011

DOI https://doi.org/10.2169/internalmedicine.0416-17

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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.

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