Grimaldi G, Reuter V, Russo P
Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Ann Surg Oncol. 1998 Sep;5(6):548-52. doi: 10.1007/BF02303649.
Bilateral renal cell carcinoma (RCC) exists in hereditary forms (von Hippel-Lindau disease, hereditary papillary renal cell carcinoma, and hereditary clear cell renal carcinoma) associated with various chromosomal abnormalities, and non-hereditary, apparently sporadic forms. The focus of this study is the clinical description of the latter entity.
Synchronous and asynchronous bilateral RCC were identified from a prospective database of 698 consecutive patients undergoing operation for RCC between July 1989 and December 1997 at Memorial Sloan-Kettering Cancer Center. Non-familial RCC was defined as that occurring in those patients without a family or hereditary history of RCC. Patients' records were evaluated for presentation, surgical approach used, and pathology. Actuarial survival from the date of initial operative treatment until the date of last follow-up or death was determined using the Kaplan-Meier method. Comparisons between groups were made using the Mann-Whitney test.
Thirty-three of 698 (4.7%) patients operated for RCC had bilateral disease. Four of the 33 (12.1%) patients had either VHL or documented hereditary RCC, and 29 of 33 (87.9%) had non-familial RCC. Of the 29 patients, histology was conventional (clear cell) in 17 patients, papillary in 5, oncocytoma in 3, and unclassified in 3. One patient had a conventional (clear cell) histology in the first nephrectomy specimen and chromophobe renal cell carcinoma in the second. Partial nephrectomy was used in 100% of patients. Median follow-up time was 52 months. Actuarial 5-year overall survival was 84.5%, and actuarial disease-specific survival was 93.3% at 5 years for the non-familial RCC patients.
Non-familial bilateral RCC patients represent a distinct subpopulation of renal cancer patients with a good overall prognosis. Partial nephrectomy is an integral part of the surgical management. Although most bilateral tumors present synchronously, asynchronous lesions may occur many years after original nephrectomy, thus committing the patient to long-term follow-up.
双侧肾细胞癌（RCC）存在与各种染色体异常相关的遗传性形式（冯希佩尔-林道病、遗传性乳头状肾细胞癌和遗传性透明细胞肾细胞癌），以及非遗传性、明显散发的形式。本研究的重点是后一种情况的临床描述。
从1989年7月至1997年12月在纪念斯隆-凯特琳癌症中心接受RCC手术的698例连续患者的前瞻性数据库中识别出同步和异步双侧RCC。非家族性RCC定义为在没有RCC家族史或遗传病史的患者中发生的RCC。评估患者记录的临床表现、所采用的手术方法和病理情况。使用Kaplan-Meier方法确定从初始手术治疗日期到最后一次随访或死亡日期的精算生存率。使用Mann-Whitney检验进行组间比较。
698例接受RCC手术的患者中有33例（4.7%）患有双侧疾病。33例患者中有4例（12.1%）患有VHL或记录在案的遗传性RCC，33例中有29例（87.9%）患有非家族性RCC。在这29例患者中，17例组织学类型为传统型（透明细胞），5例为乳头状，3例为嗜酸细胞瘤，3例未分类。1例患者在第一次肾切除标本中组织学类型为传统型（透明细胞），第二次为嫌色肾细胞癌。所有患者均采用了部分肾切除术。中位随访时间为52个月。非家族性RCC患者的5年精算总生存率为84.5%，5年精算疾病特异性生存率为93.3%。
非家族性双侧RCC患者代表了一个独特的肾癌患者亚群，总体预后良好。部分肾切除术是手术治疗的一个组成部分。虽然大多数双侧肿瘤是同步出现的，但异步病变可能在初次肾切除术后多年出现，因此患者需要长期随访。
