Clinicopathological Features and Prognosis of Renal Cell Carcinoma in Japanese Patients with von Hippel-Lindau Disease - Abstract
Purpose: We analyzed the clinicopathological features and prognosis of renal cell carcinoma (RCC) in Japanese patients with von Hippel Lindau (vHL) disease.
Patients and methods: The subject was 52 vHL patients with kidney tumors. Clinical, pathological, and survival data were collected for each patient. Overall survival was calculated from the date of initial diagnosis of kidney tumor to the date of death as a result of any cause or was censored at the date of the last follow-up. Median follow-up duration of all 52 patients was 79 months.
Results: Of 52 patients, bilateral tumor was observed in 33 patients and multifocal tumors in 40. Median tumor size of largest tumor in each patient was 3.3 cm in diameter. With regard to the treatment, most patients underwent nephron-sparing surgery. All patients had clear cell carcinoma. Although half of patients experienced local recurrences, the 10-year overall survival was 82%. At last follow-up, four patients died of RCC, 5 patients died of central nervous system hemangioblastoma, and 1 died from gastric cancer.
Conclusions: RCC in VHL patients differs from sporadic RCC in clinical features and should be carefully treated and followed closely. Appropriate decisions regarding treatment of RCC in vHL patients should be made from not only oncological outcomes but also long-term renal function outcomes and QOL.