Attainment of a long-term favorable outcome by sunitinib treatment for pancreatic neuroendocrine tumor and renal cell carcinoma associated with von Hippel-Lindau disease

Akihiro Kobayashi, Masanobu Takahashi, Hiroo Imai, Shoko Akiyama, Shunsuke Sugiyama, Keigo Komine, Ken Saijo, Masahiro Takahashi, Shin Takahashi, Hidekazu Shirota, Naomi Sato, Fumiyoshi Fujishima, Taro Shuin, Hideki Shimodaira, Chikashi Ishioka

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)

Abstract

Von Hippel-Lindau (VHL) disease, caused by germline mutations in the VHL gene, is a hereditary autosomal-dominant disorder which predisposes the individual to various malignant and benign tumors. VHL acts as a tumor suppressor, mainly through the negative regulation of hypoxia-inducible factors. Moleculartargeted drugs against vascular endothelial growth factor-signaling pathways, a target of hypoxia-inducible factors, have recently been introduced into clinical practice for the treatment of patients with sporadic renal cell carcinoma and pancreatic neuroendocrine tumors. However, whether such treatments are effective in patients with VHL disease remains to be elucidated. We herein report a Japanese patient with VHL disease who was successfully treated with sunitinib for approximately 5 years.

Original languageEnglish
Pages (from-to)629-634
Number of pages6
JournalInternal Medicine
Volume55
Issue number6
DOIs
Publication statusPublished - 2016 Mar 15

Keywords

  • Pancreatic neuroendocrine tumor
  • Renal cell carcinoma
  • Sunitinib
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Internal Medicine

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