OBJECTIVE: Von Hippel-Lindau disease (VHL) is an autosomal, dominant, hereditary disease occurring in approximately one in 36,000 births. VHL disease produces a variety of tumors and cysts in the central nervous system and visceral organs. Surgical management, when possible, improves prognosis and extends patient's life. When surgery is impossible, treatment with tyrosine kinase inhibitors demonstrates encouraging response rates.
MATERIAL AND METHOD: We present a 60-year old patient with coexistence of multifocal renal cell carcinomas (RCC) and pancreatic neuroendocrine tumor (NET) in VHL disease, who received Sunitinib as the best option of treatment.
RESULTS: Progression - free survival time is over 4 years. Regarding her acceptable tolerance for tyrosine kinase inhibitors, medical treatment is continued.
CONCLUSION: RCC and pancreatic NET associated with VHL are responsive to Sunitinib for prolonged periods of time. Tyrosine kinase inhibitors treatment for patients with multiple neoplasms associated with VHL disease may too be considered. Sunitinib showed acceptable toxicity.