title: Attainment of a Long-term Favorable Outcome by Sunitinib Treatment for Pancreatic Neuroendocrine Tumor and Renal Cell Carcinoma Associated with von Hippel-Lindau Disease.
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. Molecular-targeted 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.
authors.affiliation: Department of Clinical Oncology, Institute of Development, Aging and Cancer, Tohoku University, Japan.
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kobayashi
authors.forename: Masanobu
authors.initials: M
authors.lastname: Takahashi
authors.forename: Hiroo
authors.initials: H
authors.lastname: Imai
authors.forename: Shoko
authors.initials: S
authors.lastname: Akiyama
authors.forename: Shunsuke
authors.initials: S
authors.lastname: Sugiyama
authors.forename: Keigo
authors.initials: K
authors.lastname: Komine
authors.forename: Ken
authors.initials: K
authors.lastname: Saijo
authors.forename: Masahiro
authors.initials: M
authors.lastname: Takahashi
authors.forename: Shin
authors.initials: S
authors.lastname: Takahashi
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Shirota
authors.forename: Naomi
authors.initials: N
authors.lastname: Sato
authors.forename: Fumiyoshi
authors.initials: F
authors.lastname: Fujishima
authors.forename: Taro
authors.initials: T
authors.lastname: Shuin
authors.forename: Hideki
authors.initials: H
authors.lastname: Shimodaira
authors.forename: Chikashi
authors.initials: C
authors.lastname: Ishioka
mesh_terms: D007516:Adenoma, Islet Cell / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage* / Q000494:pharmacology; D002292:Carcinoma, Renal Cell / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D004334:Drug Administration Schedule; D017809:Fatal Outcome; D016147:Genes, Tumor Suppressor / Q000187:drug effects; D018095:Germ-Line Mutation / Q000187:drug effects; D006801:Humans; D007211:Indoles / Q000008:administration & dosage* / Q000494:pharmacology; D007680:Kidney Neoplasms / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D008297:Male; D010190:Pancreatic Neoplasms / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D011758:Pyrroles / Q000008:administration & dosage* / Q000494:pharmacology; D015398:Signal Transduction / Q000187:drug effects; D000077210:Sunitinib; D013997:Time Factors; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors*; D006623:von Hippel-Lindau Disease / Q000150:complications*
chemical_list: D000970:Antineoplastic Agents; D007211:Indoles; D011758:Pyrroles; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000077210:Sunitinib