Natural history of renal cell carcinoma associated with von Hippel-Lindau disease

Abstract

Objective To study the natural history of renal cell carcinomas associated with von Hippel-Lindau disease (VHL). Methods An active surveillance strategy was carried out on 11 VHL cases(5 males and 6 females with average age of 45 years)with 32 renal masses. The mean maximum diameter of these masses at initial diagnosis was 2. 5 cm ranging from 0. 5 to 6.2 cm. Clinical materials, radiographic, and pathologic records were reviewed to determine tumor growth rate, subsequent interventions and outcome of follow-up. Results During a median follow-up of 70 months (range 32 to 258 months), bilateral solid renal tumors developed in 6 patients;13 surgical interventions were performed in 8 cases;tumor local recurrence occurred in 4 cases;3 cases died (2 of metastasis diseases and 1 of an unrelated disease) ;8 cases survived with 1 case taking regular hemodialysis.The median follow-up duration for 32 renal masses was 51 months (range 19 to 106 months). The mean tumor growth rate observed were 0. 55 cm/year (range 0. 04 to 1.75 em/year). Three tumors grew faster (1.13-1.75 cm/year), and the other 29 tumors grew slower (0. 17-0.88 cm/year).Among the 3 tumors, 1 was found to be grade Ⅱ at pathologic examination and another developed metastasis. Progression to metastatic disease was found in 2 patients. At the last follow-up, 27 (84 %)tumors were larger than 3 cm and no metastasis disease developed among tumors less than 4 cm.Conclusions The majority of enhanced renal masses with VHL disease may still be indolent and do not metastasize during long period of follow-up even in tumors larger than 3 cm. Active surveillance appears to be a reasonable option for VHL patients with enhanced renal masses less than 4 cm. Key words: Hippel-Lindau diseases; Carcinoma, renal cell; Follow-up studies; Natural history