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

• Published in: Internal Medicine Vol. 57; no. 14; pp. 2007 - 2011
• Main Authors: Miki, Masami, Kawabe, Ken, Igarashi, Hisato, Abe, Tatsuro, Ohishi, Yoshihiro, Hashimoto, Risa, Karashima, Takashi, Yamasaki, Ichiro, Inoue, Keiji, Ito, Tetsuhide, Ogawa, Yoshihiro
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 15.07.2018; Japan Science and Technology Agency
• Subjects: Antineoplastic Agents - therapeutic use; Asian Continental Ancestry Group; Autopsies; Autopsy; Biopsy; Carcinoma, Neuroendocrine - drug therapy; Carcinoma, Neuroendocrine - etiology; Carcinoma, Neuroendocrine - physiopathology; Case Report; everolimus; Everolimus - therapeutic use; Female; Humans; Indoles - therapeutic use; Internal medicine; Liver; Liver Neoplasms - drug therapy; Liver Neoplasms - etiology; Liver Neoplasms - physiopathology; Medical treatment; Middle Aged; Neuroendocrine tumors; Pancreas; Pancreatic carcinoma; Pancreatic Neoplasms - drug therapy; Pancreatic Neoplasms - etiology; Pancreatic Neoplasms - physiopathology; pancreatic neuroendocrine tumors-G3 (NET-G3); Pyrroles - therapeutic use; Sunitinib; Treatment Outcome; Tumors; VHL protein; Von Hippel-Lindau disease; von Hippel-Lindau disease (VHL); von Hippel-Lindau Disease - complications; von Hippel-Lindau Disease - physiopathology; well-differentiated neuroendocrine carcinoma (WDNEC); WHO G3; everolimus; pancreatic neuroendocrine tumors-G3 (NET-G3); von Hippel-Lindau disease (VHL); WHO G3; well-differentiated neuroendocrine carcinoma (WDNEC)
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.0416-17

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.