Journal
INTERNAL MEDICINE
Volume 55, Issue 6, Pages 629-634Publisher
JAPAN SOC INTERNAL MEDICINE
DOI: 10.2169/internalmedicine.55.5796
Keywords
sunitinib; pancreatic neuroendocrine tumor; renal cell carcinoma; von Hippel-Lindau disease
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Funding
- Taiho
- Chugai
- Takeda
- Byer
- Pfeizer
- Mochida
- Asahi Kasei
- Bristol-Myers Squibb
- Daiichi-Sankyo
- Merk Serono
- Novartis
- Yakult
- Ono
- Grants-in-Aid for Scientific Research [16K07106] Funding Source: KAKEN
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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.
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