保留肾单位手术治疗早期肾癌的长期疗效观察

目的：探讨保留肾单位手术治疗早期肾癌的临床应用价值。方法：对采取保留肾单位手术治疗的23例孤立肾或对侧肾功能不全的早期肾癌临床资料进行回顾性分析。结果：术后均未发生严重并发症，术后病理均为T1a期，其中透明细胞癌19例，嫌色细胞癌2例，乳头状肾癌1例，囊性肾癌1例。术后平均随访44．7个月，肿瘤复发1例，复发率为4．3％。1年、3年无瘤生存率分别为100％(23／23)、93．8％(15／16)。随访期间未出现肾功能衰竭而需透析治疗者。结论：孤立肾或对侧肾功能不全的早期肾癌，是施行保留肾单位手术的绝对指征，在最大限度保留肾功能的前提下可以获得与根治性肾切除相似的疗效。     

The role of nephron sparing surgery for metastatic (pM1) renal cell carcinoma

PURPOSE: Studies have demonstrated increased time to progression when cytoreductive nephrectomy is performed for metastatic renal cell carcinoma. We evaluated the role of nephron sparing surgery in these patients. MATERIALS AND METHODS: We selected all patients with pM1 renal cell carcinoma treated with nephron sparing surgery or radical nephrectomy, and all patients with pM0 renal cell carcinoma undergoing nephron sparing surgery for solitary kidney from 1970 to 2002 from the Mayo Clinic Nephrectomy Registry. RESULTS: We identified 16 patients who underwent nephron sparing surgery for pM1 renal cell carcinoma. Solitary kidney was present in 12, 3 had bilateral synchronous disease and 1 had elective nephron sparing surgery. Cancer specific survival rates at 1, 3 and 5 years were 81%, 49% and 49%, respectively. We identified 404 patients who underwent radical nephrectomy for pM1 renal cell carcinoma. Cancer specific survival rates at 1, 3 and 5 years were 51%, 21% and 13%, respectively. The pM1 nephron sparing surgery for solitary kidney cases were more likely to have early (33% vs 10%, p = 0.009) or late (50% vs 19%, p = 0.018) complications compared with pM1 radical nephrectomy cases. There were no significant differences in early (p = 0.475) or late (p = 0.350) complications between pM1 nephron sparing surgery cases and 139 pM0 nephron sparing surgery cases. CONCLUSIONS: Cancer specific survival rates in pM1 nephron sparing surgery cases were comparable to pM1 radical nephrectomy cases. Although there were differences in early and late complications between the pM1 nephron sparing surgery and pM1 radical nephrectomy groups, there were no differences when compared with imperative pM0 nephron sparing surgery cases. This study demonstrates that nephron sparing surgery can achieve adequate cytoreductive therapy while preserving renal function, with postoperative complication rates similar to those of pM0 nephron sparing surgery cases.     

Incidental renal cell carcinoma and nephron sparing surgery

The role of nephron sparing surgery in patients with a solitary kidney, bilateral kidney tumors or an impaired renal function has been widely accepted. Partial nephrectomy in patients with a normal contralateral kidney is still under discussion. Nevertheless, more and more surgeons perform nephron sparing surgery in these patients with good results. From historical comparisons there seems to be no statistically significant difference in five-year survival between radical nephrectomy and nephron sparing surgery when the tumor diameter is four centimeters or less. The most common problem is the risk of tumor recurrence due to the multifocality. However, multifocality is more frequent than kidney recurrence, questioning the spontaneous evolution of satellite lesions. Further investigations are necessary to optimize patient selection. Therefore, a randomized prospective multicenter study with long-term follow-up might add to the excellent results published by several authors during the last two years in order to confirm that nephron sparing surgery is an excellent alternative to radical nephrectomy in small asymptomatic renal cell carcinoma.     

后腹腔镜下肾部分切除术在治疗肾脏肿瘤中的临床疗效

目的 探讨后腹腔镜下保留肾单位的肾部分切除术在治疗肾脏肿瘤的临床应用价值.方法 回顾性分析施行后腹腔镜保留肾单位的肾部分切除术的70例患者的临床资料,其中男42例,女28例,年龄平均(56±11.8)岁,肿瘤直径(3.4士1.3)cm.结果 70例患者均成功在后腹腔镜下实施手术,无1例术中中转为开放手术.手术时间100～180 min,平均(130±27)min.血管阻断时间20～40min,平均每例患者25 min.术中失血50～800mL.术后出血2例:1例发生在术后第4天,行选择性血管栓塞术后好转;另1例出现在术后第7天,经选择性血管栓塞后未见好转遂行患肾切除术.术后病检:肾透明细胞癌53例,肾乳头状癌12例,肾嫌色细胞癌2例,囊性肾癌2例,肾脏囊肿并出血1例.随访3～18个月无局部复发及远处转移.结论 后腹腔镜下保留肾单位的肾部分切除术治疗早期肾脏肿瘤安全、有效,兼有创伤小、康复快等优点,近期疗效满意,远期疗效有待进一步观察.     

Disease Outcome in Patients With Low Stage Renal Cell Carcinoma Treated With Nephron Sparing or Radical Surgery

Purpose

We investigated the outcome of nephron sparing surgery in patients with low grade and low stage (Robson stage II or less) renal cell carcinoma.

Materials and Methods

We retrospectively reviewed the records of 185 patients treated with nephron sparing surgery and 209 matched for patient age and sex, and tumor stage and grade who were treated with radical nephrectomy. Kaplan-Meier survival curves were constructed for progression and survival end points. Multivariate analysis was performed to determine the tumor characteristics independently correlated with progression and cancer death.

Results

No significant difference was observed with respect to progression-free, crude or cancer specific survival between the nephron sparing surgery and radical nephrectomy groups. Less than 5 percent of the patients treated with conservative nephron sparing surgery had local recurrence. Tumor size was a strong independent predictor of outcome, whereas Robson stage was not. Patients treated with radical nephrectomy had a significant cancer specific and progression-free survival advantages when controlling for tumor diameter and grade. However, no difference was observed in patients with primary tumor diameters of 4 cm. or less.

Conclusions

Robson staging is inaccurate in predicting tumor behavior. Patients with tumors larger than 4 cm. and a normal contralateral kidney may be best served by radical nephrectomy rather than elective nephron sparing surgery. However, nephron sparing surgery may result in an outcome similar to that of radical nephrectomy for low grade, low stage renal cell carcinomas of 4 cm. or smaller.     

肾嫌色细胞癌临床特征分析

目的 探讨肾嫌色细胞癌的临床特点并分析其预后.方法 对1998年1月至2008年1月期间收治的29例肾嫌色细胞癌患者的临床资料包括临床表现、影像学检查、治疗方法、Ptnm分期和生存期随访等,进行回顾性分析.结果 所有29例患者均缺乏典型的临床症状,术前影像学检查亦未发现特异性表现.23例患者行根治性肾切除术,6例行保留肾单位手术(NSS),病理分期:Pt1n0m0 11例,Pt2n0m0 8例,pT3aN0M0 5例,Pt1nm0 3例,Pt2n1m0 2例.26例患者获得随访,随访24～144个月,平均90个月;3例死于心脑血管疾病,6例局部复发后4例再次手术,1例远处转移,21例无瘤生存.与同期肾透明细胞癌患者相比,累积5年生存率两者分别为83.9%和63.8%,差异无统计学意义(P＞0.05),累积10年生存率分别为77.9%和49.9%,差异具有统计学意义(P＜0.01).结论 肾嫌色细胞癌是一种少见的具有特殊形态的肾细胞癌病理亚型,主要通过病理学予以鉴别诊断,手术切除是治疗初发或复发的肾嫌色细胞癌的首选方法,其预后较好.

Abstract：

Objectives To explore the clinical, pathological features and prognosis of patients with chromophobe renal cell carcinoma. Methods From January 1998 to January 2008, clinical data of 29 patients with chromophobe renal cell carcinoma including clinical manifestations, imaging examinations,treatment models, pTNM stages and follow-up results, were summarized to investigate its features and prognosis. Results All cases had no obvious clinical and preoperative imaging presentation. There were 23 patients underwent radical nephrectomy, and 6 cases underwent nephron sparing surgery. Postoperative pathological findings confirmed the diagnosis of chromophobe renal cell carcinoma. Macroscopically, the cut surface of the tumors were generally beige in color. Histologically, it showed polygonal chromophobe cells and small round eosinophilic cells with eccentric hyaline degeneration. These tumor cells had a clear and sharp membrane, lightly stained abundant cytoplasm with a fine reticular translucent pattern and irregular nuclei. And a perinuclear halo was often seen in these cells. Histochemically, the tumor cells generally show a diffuse and strong reaction for CK-8 with a negative expression of Vimentin. The pTNM stages of the tumor were as follows, pT1N0M0 in 11 cases, pT2N0M0 in 8 cases, pT3aN0M0 in 5 cases, pT1N1M0 in 3 cases,pT2N1M0 in 2 cases. Twenty-six cases of patients were followed up (24 to 144 months, with an average of 90 months), 3 cases died of cardio-cerebrovascular disease, and local recurrence involved in 6 cases with reoperation in 4 cases, as well as distant metastasis in 1 case. Twenty-one cases survived with tumor-free.The statistical results indicated that the survival rates of the patients with chromophobe renal cell carcinoma in five years and ten years were 83.9%, 77.9%, respectively, compared with renal cell carcinoma of the same stage 63. 8% and 49. 9% at the same periods, and there is no difference in the survival rate of five years( P ＞ 0. 05 ) but significant difference in that of ten years ( P ＜ 0. 01 ). Conclusions Chromophobe renal cell carcinoma is a morphologically uncommon subtype of renal cell carcinoma with the good prognosis. Definite diagnosis depends on its typical pathological feature. Radical nephrectomy is the first choice for the treatment of chromophobe renal cell carcinoma.     

保存肾单位的肾癌切除术适应证及疗效观察：附17例报告

目的 探讨保存肾单位的肾部切除术适应证,观察其治疗效果。方法 对１９９０～１９９８年施行的保存肾单位的肾癌切除术１７例患者进行回顾性分析,其中１１例作肾肿瘤切除术,６例作肾上极或下极切除术。结果 术后随访１～６．５年,除１年术后５年死于肿瘤转移外,其余均正常,预后满意。结论 对双侧同时发生无症状性肾癌、孤立肾伴肾癌或需靠双侧肾维持功能的肾癌,可考虑行保存肾单位的肾癌切除术;保存肾单位的肾癌切除术效     

小肾癌同期远处转移4例报告并文献复习

目的结合病例及文献就小肾癌同期发生远处转移相关因素、常见转移部位及诊断治疗策略做一介绍,提高对小肾癌同期发生远处转移的认识。方法回顾性分析西安交通大学第一附属医院泌尿外科肾癌患者临床资料,共发现4例小肾癌同期转移:患者均为男性,年龄40~68岁。影像学检查提示肾肿瘤直径均小于4cm,肺部、对侧肾上腺及全身多处骨骼发现转移病灶。所有患者临床诊断均为T1aN0M1。结果 4例患者分别行肾部分切除术、肾切除术、肋骨转移灶切除术、肾上腺转移瘤切除术及肾动脉栓塞术,术后给予分子靶向及细胞因子治疗;术后病理结果为透明细胞癌Ⅱ~Ⅲ级;术后随访:1例患者良好,未发现局部进展及转移病灶,1例失访,2例患者死亡,其中1例病情进展,死于多器官功能衰竭,另1例死亡原因不明。结论小肾癌同期远处转移并不罕见,特别是病理诊断为高度恶性,微血管侵犯及病理类型为肉瘤样、乳头状和嫌色细胞癌的患者。小肾癌同期发生远处转移者治疗效果远较无同期转移者差,预后主要取决远处转移部位、病理分级和病理类型。     

小肾癌54例诊治分析

目的 提高小肾癌的诊断水平和治疗效果。方法 对54例直径小于3cm的小肾癌的诊断与治疗进行回顾性分析。结果 54例中因体检或其他疾病检查时发现者37例（68．5％）；腰痛9例（16．7％）；血尿5例（0．9％）；腰痛伴血尿3例。行根治性肾切除术43例．行保留肾单位手术11例。术后均经病理证实。术中快速冰冻切片证实5例。结论 小肾癌多为偶然发现。其早期诊断主要依靠B超、CT、MRI和DSA等影像学检查；手术视具体情况行根治性肾切除术或保留肾单位手术；小肾癌病理分期低。肿瘤体积小．预后较好，生存率高。     

肾癌患者治疗方法的选择

目的:探讨肾细胞癌的不同手术方式、术前肾动脉栓塞及免疫治疗的临床应用价值。方法:回顾性分析179例肾细胞癌患者的临床资料。对患者的临床资料分组进行对比,并对治疗效果和随访结果作进一步统计学分析。结果:小肾癌行肾癌根治术与保留肾组织手术效果比较,在手术时间、术后住院时间、术后5年生存率上差异均无统计学意义(P>0.05);78例术前行选择性肾动脉栓塞者,手术证实栓塞效果满意。结论:保肾单位手术是治疗局限性小肾癌的有效手段;较大的肾癌术前进行肾动脉栓塞术便于手术切除病灶,提高了肿瘤的切除率;免疫治疗是继手术治疗之后的又一种主要临床治疗方式,尤其肿瘤疫苗的出现,对于晚期肾癌及转移癌效果明显。     

Long-term results of nephron sparing surgery for localized renal cell carcinoma: 10-year followup

PURPOSE: Partial nephrectomy is effective for renal cell carcinoma when preservation of renal function is a concern. We present the 10-year followup of patients treated with nephron sparing surgery at our institution. MATERIALS AND METHODS: Partial nephrectomy was performed in 107 patients with localized sporadic renal cell carcinoma before December 1988. Tumors were symptomatic in 73 patients (68%) and indications for surgery were imperative in 96 (90%). Of the patients 42 (39%) had renal insufficiency preoperatively. All patients were followed a minimum of 10 years or until death. RESULTS: At the end of the followup interval 32 patients (30%) had no evidence of recurrence, 28 (26%) died of metastatic renal cell carcinoma and 46 (42%) died of unrelated causes. Cancer specific survival was 88.2% at 5 and 73% at 10 years, and was significantly affected by tumor stage, symptoms, tumor laterality and tumor size. Long-term renal function was stable in 52 patients (49%). CONCLUSIONS: Partial nephrectomy is effective for localized renal cell carcinoma, providing long-term tumor control with preservation of renal function.     

肾癌保留肾单位手术的临床价值(附17例报告)

目的　评价肾癌保留肾单位手术的临床价值。　方法　回顾性研究 17例行保留肾单位手术的肾癌患者临床资料 ,其中单侧 15例 ,双肾异时性肾癌且一侧为多发肿瘤 2例。绝对指征 2例 ,相对指征 6例 ,选择性指征 9例。肿瘤直径 2～ 6cm ,均为T1期 (1997年TNM标准 ) ;行改良肾肿瘤剜除术 (切缘于肾肿瘤外 1cm正常肾实质处 ) 15例 ,肾上极切除术 1例 ,楔形切除术 1例。 2 0例同期肾癌临床及分期相当但行根治性手术的患者资料作随访对照 ,并作生存率时序检验。　结果　 17例患者随访 3～ 6 3个月 ,平均 35 .2个月 ,未见并发症及残肾肿瘤复发。无瘤生存率与根治性手术者相近。　结论　肾癌保留肾单位手术安全、有效 ,适合于对侧肾功能正常、一侧局限的偶发肾肿瘤患者。     

Nephron sparing surgery for renal tumors: indications, techniques and outcomes.

PURPOSE: A contemporary review of the indications, techniques and outcomes is presented for nephron sparing approaches to solid renal masses, emphasizing their role for the treatment of renal cell carcinoma. We also reviewed the evolving role of minimally invasive forms of parenchymal sparing renal surgery. MATERIALS AND METHODS: MEDLINE and CANCERLIT computerized literature searches, and manual bibliographic reviews were performed to identify published peer reviewed articles pertaining to nephron sparing surgery or partial nephrectomy from 1980 to 2000. Pertinent articles were collated and reviewed. RESULTS: Nephron sparing surgery is increasingly being used to treat patients with solid renal lesions. The technical success rate of nephron sparing surgery is excellent, and operative morbidity and mortality are low. For renal cell carcinoma long-term cancer-free survival is comparable to that after radical nephrectomy, particularly for low stage disease. The overall incidence of local recurrence is low at 0% to 10%. For tumors 4 cm. or less local recurrence rates are even less at 0% to 3%. The risk of local recurrence depends primarily on the initial local pathological tumor stage. The reported incidence of multifocal renal cell carcinoma is approximately 15% and it also depends on tumor size, histology and stage. The risk of multifocal disease is low at less than 5% when the maximal diameter of the primary tumor is 4 cm. or less. Recent advances in renal imaging limit the radiographic evaluation necessary when planning complex nephron sparing approaches. Three-dimensional, volume rendered computerized tomography integrates all of the necessary information previously obtained by conventional computerized tomography, angiography, venography and pyelography into a single preoperative test, allowing better operative planning with maximal preservation of unaffected parenchyma in the remnant kidney. Minimally invasive modalities of tumor resection or destruction should be reserved for highly select patients and await improvements in technology, standardization of technique and long-term outcomes data before they may be completely integrated options. CONCLUSIONS: Nephron sparing surgery provides effective therapy for patients in whom preservation of renal function is a relevant clinical consideration. The importance of meticulous operative technique for achieving acceptable oncological and functional outcomes is emphasized. Accumulating data in appropriately select patients suggest a long-term functional advantage gained by the maximal preservation of unaffected renal parenchyma without sacrificing cancer control.     

肾细胞癌271例临床分析

目的 探讨肾细胞癌的诊治方法.方法 1993年1月至2000年12月共收治肾细胞癌患者271例,对诊断方法、治疗手段、随访及预后进行回顾性分析.结果 B超、CT仍为主要的诊断方法.手术治疗234例患者,其中行根治性肾切除术197例(72.6%),保留肾单位手术19例;转移瘤切除术6例;其他手术12例.病理结果:透明细胞癌137例,占61.4%(137/223)、颗粒细胞癌18例(8.1%)、混合细胞癌32例(14.3%)、乳头状腺癌23例(10.3%),其他13例.210例患者(77.5%)获得随访.1、3、5、10年生存率分别为95.3%(182/191)、88.7%(107/122)、74.7%(56/75)和32.1%(10/31).结论 B超是检测肾细胞癌的首选检查方法,CT为最有价值诊断方法.提高肾细胞癌远期生存率最关键的因素是早期发现、早期行肾癌根治术或保留肾单位的肾癌切除术.     

保留肾单位手术治疗小肾癌的可行性与疗效评估

目的:探讨保留肾单位手术(NSS)治疗小肾癌的可行性和疗效.方法:报告20例行NSS治疗小肾癌患者(NSS组)和21例和根治性肾切除术(RN)治疗的同种患者(RN组)的临床资料,比较两组的手术时间、术中出血量、术后住院时间、并发症、复发情况及存活率.结果:两组手术均顺利完成.NSS组平均手术时间、术中出血量、并发症发生率大于RN组(P<0.05);但两组间术后住院时间、肿瘤复发率及长期存活率方面,差异无统计学意义.结论:NSS切除治疗小肾癌具有安全有效性.但要严格掌握适应证,对于大于4cm的肾癌,尽可能行RN.     

Nephron-sparing surgery--strategies for partial nephrectomy in renal cell carcinoma.

The use of partial nephrectomy for renal cell carcinoma has continuously changed in the clinical practice. Previously it was mostly used in imperative cases, in patients with a solitary kidney or in patients with a risk of renal failure. An increased number of incidentally detected renal cell carcinomas are diagnosed due to the advances of the radiological methods. These tumours tend to be smaller and generally with a lower stage. The reported excellent results of partial nephrectomy have promoted the use of nephron-sparing surgery also in patients with a normal contralateral kidney and tumours smaller than 4-5 cm. The technical outcome is excellent with a low operative morbidity and a good oncologic control. Therefore partial nephrectomy has become a standard technique in the treatment of properly selected patients. Laparoscopy with its reduced postoperative pain and shorter rehabilitation time, has encouraged the interest in minimally invasive nephron sparing surgical techniques. Although low, the risk of local tumour recurrence and surgical complications are higher after nephron-sparing surgery compared with radical nephrectomy. Furthermore, long-term renal function remains adequate in most patients with a normally functioning contralateral kidney also after radical nephrectomy. Albeit these facts, there is convincing evidence justifying nephron-sparing surgery to be used routinely for patients with a small renal cell carcinoma and a normal functioning contralateral kidney.     

肾癌肾部分切除术的临床价值及合适的手术切缘的探讨

目的：探讨肾癌肾部分切除术(保留肾单位手术)的临床价值及合适的手术切缘。方法：回顾性分析15例行肾部分切除术的肾癌患者临床资料．其中双侧异时性肾癌且一侧为多发肿瘤2例，单发肿瘤13例。肿瘤直径2～6cm．均为T1期(1997年TNM分期标准)。对15例肾癌患者行肾部分切除术．手术切缘位于肿瘤外1cm。另取肾癌根治性手术标本21例．于体外沿假包膜行肾肿瘤剜除术．并随机切取肿瘤边缘0．3cm、0．5cm及1cm处肾实质及肾蒂处淋巴脂肪组织行病理检查。结果：15冽患者随访12～72个月．平均41个月．未见并发症及残肾内肿瘤复发。21例标本于体外行肿瘤剜除后肉眼下均无肿瘤组织残留，送检组织均无肿瘤细胞浸润。结论：肾部分切除术能安全有效地治疗局限的早期肾癌患者．而手术切缘为肿瘤边缘1cm处较为合适。     

56例肾细胞癌手术治疗的临床分析

目的：探讨肾细胞癌（肾癌）的手术方式,以进一步提高其疗效。方法：对56例肾癌手术治疗的临床资料进行回顾性分析,实施根治性肾切除术者48例,其中4例同时进行下腔静脉癌栓切除术;实施保留肾单位手术者3例;实施其他类型手术者5例。结果：病理类型为透明细胞癌40例,颗粒细胞癌10例,混合型癌6例。42例获得随访,其中3年、5年和10年生存率分别为71.4%(30/42) 、54.2%(13/24)和20.0%(2/10)。结论：根治性肾切除术仍是目前 肾癌最有效的治疗方法,保留肾单位手术应严格掌握手术适应证。     

Nephron sparing surgery for central renal tumors: experience with 33 cases

PURPOSE: Nephron sparing surgery is standard treatment for small, peripherally located renal cell carcinoma. In patients with a solitary kidney, bilateral tumors or impaired renal function nephron sparing surgery provides the only option to nephrectomy and subsequent hemodialysis or transplantation. We retrospectively investigated the value of nephron sparing surgery for centrally located renal cell carcinoma. MATERIALS AND METHODS: Between 1969 and 1997, 311 renal tumor enucleations were performed at our institution. The tumor was centrally located in 33 cases. The indication for enucleation was elective in 7 cases and imperative in 26, including bilateral tumor in 16 (metachronous in 9 and synchronous in 7), chronic renal failure in 4 and solitary kidney in 6. Four patients had metastasis at enucleation. RESULTS: Convalescence was unremarkable in 28 cases. Hemorrhage occurred in 1 patient, a urinary fistula in 2 and a local abscess secondary to a urinary fistula in 1. One patient died postoperatively of heart failure. Average serum creatinine was 1.25, 1.63 and 1.33 mg./dl. preoperatively, at hospital discharge and at a mean followup of 33 months, respectively. Hemodialysis was necessary transiently during convalescence in 1 patient and permanently starting 6 years after enucleation in another. Definitive histology revealed oncocytoma in 4 cases and renal cell carcinoma in 29. Disease was stages pT1 to pT3 in 9, 18 and 2 cases, and grades 1 to 3 in 6, 18 and 5, respectively. Local recurrence developed in 2 patients. Mean followup was 5.2 years (range 0.3 to 16.7). At a mean followup of 6.2 years (range 0.7 to 16.7) 20 patients were free of disease. In addition to the patient who died postoperatively, 9 died of renal cell carcinoma at a mean of 1.6 years (range 0.3 to 5.3) and 3 died of other causes at 5, 11 and 12 years postoperatively, respectively. No patient who underwent elective enucleation died. CONCLUSIONS: Nephron sparing surgery for centrally located kidney tumors is technically feasible and associated with an acceptable complication rate. Local tumor control is excellent, and the overall prognosis depends on contralateral disease and metastasis. Benign tumors may be diagnosed and removed without loss of the kidney. By avoiding hemodialysis quality of life is improved.     

Treatment of Renal Cell Carcinoma in Von Hippel-Lindau Disease: A Multicenter Study

A total of 65 patients with von Hippel-Lindau disease underwent surgery for renal cell carcinoma (54 bilaterally and 11 unilaterally) at 8 medical centers. Only 1 patient presented with metastatic disease. Radical nephrectomy and nephron sparing surgery were performed in 16 and 49 patients, respectively. Mean posttreatment followup was 68 months.

The 5 and 10-year cancer-specific survival rates for all patients were 95 percent and 77 percent, respectively. The corresponding rates for patients treated with nephron sparing surgery were 100 percent and 81 percent, respectively. Of the latter patients 25 (51 percent) had postoperative local tumor recurrence but only 2 had concomitant metastatic disease. Survival free of local recurrence was 71 percent at 5 years but only 15 percent at 10 years. End stage renal failure occurred in 15 patients (23 percent): 6 underwent renal transplantation (5 are alive with satisfactory renal function and no evidence of malignancy) and 9 were treated with chronic dialysis (6 are free of tumor).

Our results indicate that nephron sparing surgery can provide effective initial treatment for patients with renal cell carcinoma and von Hippel-Lindau disease. These patients must be followed closely, since most will eventually have locally recurrent recurrent renal cell carcinoma. When removal of all renal tissue is necessary to achieve control of malignancy, renal transplantation can provide satisfactory replacement therapy for end stage renal disease.