von Hippel-Lindau病肾癌的自然病程研究

目的 探讨von Hippel-Lindau(VHL)病肾癌的自然病程. 方法 初诊后未立即手术的VHL病肾癌患者11例,男5例,女6例,平均年龄45岁.共32个肿瘤,肿瘤直径平均2.5(0.5～6.2)cm.采用积极监测策略对其进行临床管理.对患者临床资料、随访结果及肿瘤生长速度进行分析. 结果 中位随访时间70(32～258)个月.双肾实性占位6例;行手术治疗8例,包括保留肾单位手术7次,根治性肾切除6次;肿瘤复发4例.死于肿瘤转移2例,死于非肿瘤因素1例.32个肿瘤中位随访时间51(19～106)个月.肿瘤平均生长速度0.55 cm/年;直径＞3 cm、≤3 cm肿瘤生长速度分别为0.55、0.44 cm/年,差异无统计学意义(P=0.40).肿瘤生长快3个(1.13～1.75cm/年),肿瘤生长慢29个(0.17～0.88 cm/年).肿瘤进展转移2例.随访结束时,肿瘤＞3 cm者27个(84%),直径≤4 cm肿瘤未发现转移.结论 多数VHL病肾癌生长缓慢,不发生转移;肿瘤直径≤4 cm者很少转移;对≤4 cm VHL病肾癌采用积极监测策略可行.

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

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.