Von Hippel—Lindau病肾癌的临床特征分析

目的 探讨Von Hippel-Lindau（VHL）病肾癌的临床特点。方法回顾分析28例VHL病肾癌患者的临床资料。就初诊年龄、肿瘤部位、同时或异时癌、肿瘤的组织病理等与散发性肾癌进行比较。结果VHL肾癌初诊年龄44．6岁，双肾癌15例、多灶性肾癌16例、伴双侧多发肾囊肿20例。共切除87个实性肿瘤。术后病理：透明细胞癌86个，Fuhrman分级Ⅰ级73个、Ⅱ级12个、Ⅲ级1个；钙化结节1个。TNM分期ⅠA期、ⅠB期、Ⅱ期、Ⅲ期分别为8例、7例、8例和1例。与散发性肾癌组相比，VHL病肾癌组患者发病年龄早（P〈0．05），双肾多灶性肾癌及伴双侧多发肾囊肿比例高（P〈O．001），高级别肿瘤比例低（P〈O．05）。结论VHL病肾癌不同于散发性肾癌，有其独特的临床病理特征，这对该病诊断治疗具有一定指导价值。     

Inherited forms of renal cell carcinoma.

It is estimated that up to 2% of renal cell cancer (RCC) clusters in families. Several forms of hereditary RCC have been characterized with specific clinical, histopathological, and genetic features. The most common of these is von Hippel-Lindau (VHL) disease caused by mutations in the VHL gene and predisposing to clear cell RCC. Predisposition to papillary RCC is present in hereditary leiomyomatosis and renal cell cancer (HLRCC) and hereditary papillary renal cell carcinoma (HPRC). Identification of the genetic defects causing these diseases has enlightened the molecular pathogenesis of RCC, and moreover, provided means to improve patient management. Genetic testing enables early diagnosis of the disease, after which individuals at-risk can be guided to regular surveillance. Screening facilitates detection of presymptomatic early tumors broadening treatment options and potentially improving prognosis. Thus, identification of individuals with inherited cancer susceptibility is important as special management of these patients improves disease outcome. The purpose of this review is to provide clues for identification and management of hereditary renal cancer patients in clinical practice.     

Clear cell papillary cystadenoma of the epididymis and mesosalpinx: immunohistochemical differentiation from metastatic clear cell renal cell carcinoma

Clear cell papillary cystadenoma is a rare epithelial tumor of the epididymis, which may present as an isolated lesion or as a component of von Hippel-Lindau disease (VHLD). Recently, tumors have also been described in the female genital tract with similar histology. Recognition of clear cell papillary cystadenoma is critical because of its association with VHLD and its potential diagnostic confusion with metastatic renal cell carcinoma because of a shared architecture and clear cells. In this study, we report on the immunohistochemical differentiation of 5 clear cell papillary cystadenomas, 3 of the epididymis and 2 of the mesosalpinx, from metastatic renal cell carcinoma. In 2 cases, there was a history of renal cell carcinoma in the setting of VHLD; and in 1 of these cases, an epididymal papillary cystadenoma was initially considered to be metastatic renal cell carcinoma. Immunohistochemically, tumor cells were moderately intensely positive for cytokeratin AE1/AE3 and epithelial membrane antigen, strongly positive for CK7 and negative for CK20 and RCC. Four of 5 cases were negative for CD10. This staining profile contrasts with that reported for clear cell renal cell carcinomas, which are typically negative for CK7 and immunoreactive for renal cell carcinoma (RCC) and CD10. Our findings indicate that, in cases where there is uncertainty about the histologic diagnosis of clear cell papillary cystadenoma, the above immunohistochemical panel helps to rule out metastatic renal cell carcinoma.     

Molecular genetic mechanism of hereditary human kidney cancer development]

Here we reviewed the molecular genetic mechanism in the development of 4 types of human hereditary kidney cancers. These include von Hippel-Lindau (VHL) disease, hereditary papillary renal carcinoma, familial renal cancers with translocation of chromosome 3, and Tuberous sclerosis. Loss of function of the VHL disease gene is responsible for the von Hippel-Lindau disease and major portion of sporadic clear cell renal carcinoma. Activated c-Met oncogene is responsible for the development in some cases of hereditary papillary renal cell carcinomas and sporadic papillary renal carcinomas. There are several cases of familial renal carcinoma in that translocations of chromosome 3 p are demonstrated. The molecular genetic mechanism of this disease is not known. Several reports show the development of renal cell carcinoma in Tuberous sclerosis patients. TSC 1 or TSC 2 gene may be responsible for these tumors. The detail in this disease not well known. Molecular genetic analyses for hereditary renal cancer identified several oncogenes and tumor suppressor genes in hereditary as well as sporadic renal carcinomas. Future studies may reveal new category of oncogenes or tumor suppressor genes that are involved in the human kidney cancer development.     

Mechanisms of disease: hereditary leiomyomatosis and renal cell cancer--a distinct form of hereditary kidney cancer

Renal cell carcinoma (RCC) represents a group of diseases linked by their primary site of origin, the kidney. Studies of families with a genetic predisposition to the development of kidney cancer have revealed that multiple genes are involved in the molecular pathogenesis of RCC. Germline mutations in a gene that encodes a Krebs cycle enzyme have been found to result in a distinct clinical entity referred to as hereditary leiomyomatosis and renal cell cancer (HLRCC). HLRCC is inherited in an autosomal-dominant fashion. Affected individuals in HLRCC families are at risk for the development of leiomyomas of the skin and uterus as well as renal cancers. HLRCC-associated kidney tumors are often biologically aggressive. Linkage analysis has identified germline alterations in the fumarate hydratase (FH) gene associated with HLRCC. While the mechanisms of molecular carcinogenesis are not entirely understood, several lines of evidence derived from clinical and basic research suggest that pseudohypoxia might drive cellular transformation. The role of FH mutations in sporadic tumors seems to be limited. Nevertheless, continued investigation of HLRCC should provide further insight into the mechanisms of kidney cancer development, and could potentially identify targets for new therapeutic approaches to RCC.     

Multiple/bilateral renal tumors in patients with Birt–Hogg–Dubé syndrome

Objective To describe our findings in four patients with multiple/bilateral renal cell carcinoma (RCC) and Birt–Hogg–Dubé (BHD) syndrome. Patients and methods A series of four patients with BHD syndrome and RCC is analyzed. Patient charts were reviewed for age, sex, presentation, various clinical manifestations, imaging, management and outcome. Results Patients included 2 males and 2 females. Age ranged from 40 to 65 years (mean 56 years). The interval between the diagnosis of skin lesions characteristic of the disease and the development of renal tumors ranged between 1 and 35 years. Three of the patients had bilateral renal tumors (2 synchronous and one metachronous), one patient had multiple renal tumors in one kidney. In one patient the renal mass was diagnosed with a screening CT scan of the abdomen after the diagnosis of BHD syndrome. One patient had associated spontaneous pneumothorax and thyroid tumor. Only one of the 4 patients had prior family history of BHD syndrome. Renal tumors were clear cell type in 3 patients, and chromophobe tumor in one. Tumor size ranged from 2 to 9 cm. Conclusion BHD syndrome is associated with multiple diseases and tumors. We describe four patients with BHD syndrome with multiple or bilateral RCC. Two of the patients were asymptomatic. A high index of suspicion should be present in patients who present with the characteristic skin lesions of BHD syndrome and screening for the presence of renal tumors should be done in those patients. Long term follow up is necessary after treating renal tumors in these patients.     

Novedades etiopatogénicas en el carcinoma de células renales: carcinogénesis y vías de desarrollo tumoral

Renal cancer is the third leading urological tumor after prostate and bladder cancers. Annual incidence of renal cancer in all stages has markedly increased in recent years. This represents a true increase in the number of actual cases that is not fully accounted for by widespread use of diagnostic imaging tests.This article is intended to provide an update on the carcinogenesis and tumor development pathways involved in the genesis of this tumor. Renal cell carcinoma (RCC) arises in renal epithelium and accounts for more than 90% of all malignant kidney tumors. Approximately 2% of RCCs are associated to hereditary syndromes, specific oncogenes, or changes in tumor suppressor genes. Changes in the VHL gene exist in all hereditary cases. This gene (located in the short arm of chromosome 3:3p25-26) is also involved in more than 60% of sporadic cases. This paper systematically addresses the latest findings on implications of the VHL gene in angiogenesis and its potential relationship to new molecules involved in management of RCC.     

Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience

PURPOSE: von Hippel-Lindau disease, hereditary papillary renal cell carcinoma, the Birt-Hogg-Dubé syndrome and familial renal oncocytoma are familial renal tumor syndromes. These hereditary disorders are noteworthy for the development of multiple bilateral renal tumors and the risk of new tumors throughout life. One management strategy is observation of solid renal tumors until reaching 3 cm, then performing parenchymal sparing surgery. We present a 5-year update on our experience. MATERIALS AND METHODS: From May 1988 to October 1998, 49 patients with hereditary renal cell carcinoma, including von Hippel-Lindau disease in 44, hereditary papillary renal cell carcinoma in 4 and the Birt-Hogg-Dubé syndrome in 1, and 1 with familial renal oncocytoma underwent exploration to attempt renal parenchymal sparing surgery. Patients were followed prospectively with periodic screening for recurrence, metastasis and loss of renal function. Median followup was 79.5 months (range 0.7 to 205). RESULTS: A total of 50 patients underwent 71 operations resulting in unilateral nephrectomy in 6, bilateral nephrectomy in 1 and partial nephrectomy in 65, with 1 to 51 tumors removed from each kidney (mean 14.7). Mean patient age was 39.5 years (range 18 to 70). Of the 65 (40%) partial nephrectomies 26 were performed with cold renal ischemia. Mean blood loss was 2.9 +/- 0.5 l (range 0.15 to 23). Postoperative complications included renal atrophy in 3 patients. Mean preoperative serum creatinine was 1.05 +/- 0.03 mg/dl (range 0.6 to 1.8), and postoperative creatinine was 1.06 +/- 0.04 mg/dl (range 0.6 to 2.0). No patient who underwent renal parenchymal sparing surgery required renal replacement therapy. Metastatic disease developed in 1 patient with a 4.5 cm renal tumor. CONCLUSIONS: Parenchymal sparing surgery with a 3 cm threshold in patients with hereditary renal cancer appears to be an effective therapeutic option to maximize renal function while minimizing the risk of metastatic disease.     

Birt–Hogg–Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome

Birt–Hogg–Dubé syndrome is an autosomal dominantly inherited disease that predisposes patients to develop fibrofolliculoma, lung cysts and bilateral multifocal renal tumors, histologically hybrid oncocytic/chromophobe tumors, chromophobe renal cell carcinoma, oncocytoma, papillary renal cell carcinoma and clear cell renal cell carcinoma. The predominant forms of Birt–Hogg–Dubé syndrome‐associated renal tumors, hybrid oncocytic/chromophobe tumors and chromophobe renal cell carcinoma are typically less aggressive, and a therapeutic principle for these tumors is a surgical removal with nephron‐sparing. The timing of surgery is the most critical element for postoperative renal function, which is one of the important prognostic factors for Birt–Hogg–Dubé syndrome patients. The folliculin gene (FLCN) that is responsible for Birt–Hogg–Dubé syndrome was isolated as a novel tumor suppressor for kidney cancer. Recent studies using murine models for FLCN, a protein encoded by the FLCN gene, and its two binding partners, folliculin‐interacting protein 1 (FNIP1) and folliculin‐interacting protein 2 (FNIP2), have uncovered important roles for FLCN, FNIP1 and FNIP2 in cell metabolism, which include AMP‐activated protein kinase‐mediated energy sensing, Ppargc1a‐driven mitochondrial oxidative phosphorylation and mTORC1‐dependent cell proliferation. Birt–Hogg–Dubé syndrome is a hereditary hamartoma syndrome, which is triggered by metabolic alterations under a functional loss of FLCN/FNIP1/FNIP2 complex, a critical regulator of kidney cell proliferation rate; a mechanistic insight into the FLCN/FNIP1/FNIP2 pathway could provide us a basis for developing new therapeutics for kidney cancer.     

上海仁济医院肾癌数据库资料分析

目的 探讨肾癌临床、病理、分期、分级与预后特征. 方法 分析2003年至2005年上海仁济医院泌尿科肾癌数据库435例患者临床和病理资料.采用WHO 1997年肾实质上皮性肿瘤组织学分类标准、2002年ATCC的TNM分期和临床分期、1982年Fuhrman病理分级.采用Kaplan-Meier法和Logrank检验对57例获随访的晚期患者行生存分析和预后因素判断. 结果 435例患者中,遗传性VHL病肾癌10例(2.4%)、散发性肾透明细胞癌372例(85.5%)、乳头状癌13例(3.0%)、嫌色细胞癌18例(4.1%)、集合管癌4例(0.9%)、嗜酸性细胞腺瘤4例(0.9 %)、未分类肾癌.14例(3.2%).行根治性肾切除术335例(77.0%),保留肾单位手术74例(17.0%),姑息性肾切除等手术26例(6.0%).遗传性VHL病肾癌均为双肾癌伴多发囊肿,临床分期Ⅰ期7例、Ⅱ期3例,病理分级Ⅰ级6例、Ⅱ级4例,基因测序均存在VHL基因突变,平均随访28.6个月,患者无肿瘤局部进展或转移,但4例患者出现同侧或双侧肿瘤再发.嫌色细胞癌临床分期均为Ⅰ期,病理分级Ⅰ级5例,Ⅱ级13例,平均随访19.8个月均存活,无肿瘤转移或复发.集合管癌临床分期均为Ⅰ期,病理分级均为Ⅲ级,平均生存时间11.3个月.肾透明细胞癌和乳头状癌临床分期Ⅰ期260例(67.6%)、Ⅱ期64例(16.6%)、Ⅲ期32例(8.3%)、Ⅳ期29例(7.5%),其中T1a 147例(38.2%)、T1b 113例(29.4 %);病理分级Ⅰ级124例(32.2%)、Ⅱ级219例(56.9%)、Ⅲ级40例(10.4%)、Ⅳ级2例(0.5%).57例晚期肾癌患者中位生存时间(16.0±1.3)个月,1年生存率55.0%,2年生存率31.0%.预后因素分析显示,临床分期、肿瘤大小、淋巴结转移、远处转移和病理分级是晚期肾癌解剖水平和组织学水平的预后影响因素. 结论 不同组织学亚型的肾癌生物学特征存在较大差异,遗传性VHL病肾癌存在基因突变,常为双侧、多中心、低Fuhrman分级透明细胞癌,易再发不易转移.肾嫌色细胞癌预后较好,而集合管癌预后差.在解剖水平和组织学水平,TNM分期、肿瘤大小、淋巴结转移、远处转移和肾癌病理分级是晚期肾癌的预后影响因素.     

Emerging Entities in Renal Neoplasia

This article reviews emerging entities in renal epithelial neoplasia, including tubulocystic carcinoma, clear-cell–papillary renal cell carcinoma (RCC), thyroid-like follicular RCC, ALK-related RCC, translocation RCC, acquired cystic disease–related RCC, succinate dehydrogenase–deficient RCC, and hereditary leiomyomatosis-RCC syndrome–associated RCC. Many of these rarer subtypes of RCC were recently studied in more depth and are included in the upcoming version of the World Health Organization classification of tumors. Emphasis is placed on common gross and morphologic features, differential diagnoses, use of ancillary studies for making accurate diagnoses, molecular alterations, and predicted biologic behavior based on previous studies.     

The surgical approach to multifocal renal cancers: hereditary syndromes, ipsilateral multifocality, and bilateral tumors

Although the management of sporadic renal tumors is challenging enough, dealing with those with bilateral, multifocal, and hereditary kidney cancer adds an additional level of complexity. A clinician managing this patient population must understand the hereditary syndromes and the genetic testing available. Treating physicians must be familiar with enucleative surgery, complex or multiple tumor partial nephrectomy, complex renal reconstruction, re-operative renal surgery, and active surveillance strategies. With proper management, most patients affected with bilateral, multifocal, or hereditary RCC can have a long life expectancy while maintaining adequate renal function.     

How accurate is diagnostic imaging in determination of size and multifocality of renal cell carcinoma as a prerequisite for nephron-sparing surgery?

Background: The indication for elective nephron-sparing surgery (NSS) in renal cell carcinoma (RCC) is under discussion in the urological literature. The main problem of NSS is the multifocality of RCC. The presented study was preformed to assess the accuracy of pre-and intraoperative ultrasound (US), and computerized tomography (CT) in determination of tumor size and detection of multifocal lesions.Materials and methods: Tumor size was measured by preoperative US and CT and compared with the tumor diameters in gross sections of the neoplastic kidneys. Multifocality was determined by 3-mm step sectioning of the nephrectomy specimen, and the results were correlated with preoperative US and CT on the one hand, and the ex situ sonography of the nephrectomized kidney on the other hand.Results: US and CT show similar results in the determination of the tumor size. In only 22.9%, preoperative US and CT were able to detect multifocal tumors. Ex situ sonography had a sensitivity of 40.0% and a specificity of 87.2% in this regard.Conclusions: In preparation for nephron-sparing surgery of renal cell carcinoma, neither preoperative routine imaging, nor intraoperative ultrasound can safely predict multifocal lesions of renal cell carcinoma.CommentaryLocal tumor recurrence following nephron-sparing surgery (NSS) for renal cell carcinoma (RCC) may be due to incomplete resection of the primary tumor, occult multicentric disease or the development of a new primary or metastatic focus of RCC in the renal remnant. The risk of multicentric disease in RCC has been evaluated and debated extensively in the literature. RCC generally occurs as a discrete focal lesion rather than an infiltrative process which is seen in carcinoma of the prostate. At issue is whether the molecular events that give rise to malignant transformation affect a discrete segment of the kidney or a broader segment of the renal tubular epithelium. A high incidence of multicentric RCC has been reported in patients with germ line mutations such as those that exist in von Hippel Lindau disease and other forms of hereditary RCC suggesting a global predisposition to malignant degeneration throughout the entire renal parenchyma.The incidence of multicentricity in sporadic RCC has been less clear. Emerging cytogenetic and molecular data suggest that satellite lesions may occasionally arise from the same malignant clone as their corresponding primary lesion and may therefore represent biologically significant intra-renal metastasises. A recent review of published studies comprising over 1100 cases of sporadic RCC indicated an aggregate incidence of 15.2% of tumor multifocality (range 6.5%–28%)[1]. It is important to remember that these studies represent a diverse group of patients and that RCC is in fact a heterogenous group of tumors. The risk of multicentricity is not equal in all patients and appears to be related to other prognostic variables such as tumor histology, stage and grade. For example, papillary RCC is known to be associated with a higher incidence of multifocality than the more common clear cell variant. The risk of multifocal disease also increases with larger tumors, particularly those that extend beyond the renal capsule (pT3+). Finally, some microfocal tumors are of unknown biological significance such as the finding of satellite adenomas. Of importance when considering relative indications for elective NSS is the incidence of multifocality when the primary or index tumor is ⩽ 4 cm. A recent review of the literature indicated that the incidence of multifocality in this setting is approximately 5%.The most worrisome implication of multifocal RCC is that this will predispose to an increased risk of local tumor recurrence following NSS. Although this potential risk must be considered, the relationship between multifocality and local recurrence is neither linear or predictable as suggested by the low overall local recurrence rates reported following NSS in several large series. In nearly 1800 cases of NSS reported in the literature to date, the risk of local tumor recurrence has ranged from 0–10% and is clearly lowest among patients undergoing elective NSS for small (⩽ 4 cm) low stage lesions [1]. The true biologic significance of multicentric renal tumors and their implication for NSS therefore remain to be fully elucidated.[1] Uzzo RG, Novick AC. Nephron-sparing surgery for renal tumors: indications, techniques and outcomes. J Urol 2001;166:6–18.Andrew C. Novick, M.D.     

An unusual case of Von Hipple Lindau (VHL) syndrome with bilateral mutlicentric renal cell carcinoma with synchronous solitary urinary bladder metastasis

In VHL syndrome, renal cell carcinoma is distinctive for its early age of onset, for its bilateral and multifocal involvement. Synchronous solitary urinary bladder metastasis from renal cell carcinoma is extremely rare. We report an unusual case of VHL with bilateral multicentric renal cell carcinoma and synchronous solitary urinary bladder metastasis. To the best of our knowledge this is the first case reported in literature with this combination.     

Hereditary leiomyomatosis and renal cell cancer: a syndrome associated with an aggressive form of inherited renal cancer

PURPOSE: Hereditary leiomyomatosis and renal cell cancer is a recently described hereditary cancer syndrome in which affected individuals are at risk for cutaneous and uterine leiomyomas, and kidney cancer. Our initial experience revealed the aggressive behavior of these renal tumors, often with early metastasis, despite small primary tumor size. We report the clinical characteristics and urological treatment of patients with hereditary leiomyomatosis and renal cell cancer associated renal tumors. MATERIALS AND METHODS: A total of 19 patients with hereditary leiomyomatosis and renal cell cancer associated renal tumors were evaluated. The 11 women and 8 men had a median age at diagnosis of 39 years (range 22 to 67), and a median clinical and radiological followup of 34 months (range 6 to 141). Hereditary leiomyomatosis and renal cell cancer manifestations in patients with renal tumors included cutaneous leiomyomas in 11 of 17 evaluable patients (65%) and uterine leiomyomas in 7 of 7 evaluable females (100%). RESULTS: Median pathological tumor size was 7.8 cm (range 1.5 to 20). Histological subtypes were consistent with hereditary leiomyomatosis and renal cell cancer renal carcinoma. Four of 7 patients with 2.0 to 6.7 cm T1 tumors had spread to regional lymph nodes or metastases at nephrectomy. Overall 9 of 19 patients (47%) presented with nodal or distant metastases. CONCLUSIONS: Renal tumors in patients with hereditary leiomyomatosis and renal cell cancer syndrome are significantly more aggressive than those in patients with other hereditary renal tumor syndromes. In contrast to other familial renal cancer syndromes, the observation of 3 cm or less renal tumors associated with hereditary leiomyomatosis and renal cell cancer is not recommended. Careful followup of affected and at risk individuals in families is necessary.     

Prevalence of microscopic tumors in normal appearing renal parenchyma of patients with hereditary papillary renal cancer

PURPOSE: We describe the earliest renal lesions associated with hereditary papillary renal cancer and estimate the prevalence of microscopic papillary renal tumors. MATERIALS AND METHODS: Grossly normal tissue was obtained from 12 kidneys during renal surgery in 9 patients with hereditary papillary renal cancer. Tissue was examined microscopically and findings were compared to those previously reported to be associated with von Hippel-Lindau disease and sporadic renal cell carcinoma. RESULTS: A total of 92 microscopic papillary renal cell carcinoma lesions were identified on 46 of 88 slides (53%). No other lesions were identified. All tumors were solid and displayed the basophilic papillary histology characteristic of hereditary papillary renal cancer. Extrapolation of the data predicted the prevalence of 1,100 to 3,400 microscopic papillary tumors in a single kidney in a patient with hereditary papillary renal cancer. CONCLUSIONS: The basophilic papillary histology characteristic of clinically apparent renal tumors in patients with hereditary papillary renal cancer also characterizes the multiple microscopic lesions seen in the kidneys. These findings suggest that the earliest renal tumor in patients with an activating hereditary mutation of the met gene is papillary basophilic renal cancer. The large number of microscopic tumors in patients with hereditary papillary renal cancer was comparable to or greater than that seen in those with von Hippel-Lindau disease.     

遗传性肾癌11例临床分析

目的 探讨遗传性肾癌的诊断和治疗方法.方法 回顾性分析11例遗传性肾癌患者的临床资料,其中男8例、女3例,年龄32～67岁,平均48岁;4例为双侧肾癌,4例为多发肾癌.2例诊断为希佩尔-林道病综合征,6例诊断为家族性肾透明细胞癌,3例诊断为遗传性乳头状肾癌.10例患者行保留肾单位的手术和（或）肾癌根治术,1例未手术.结果 随访12～114个月,4例发生肿瘤复发,1例死于肿瘤转移,2例死于其他原因,4例无瘤生存.结论 遗传性肾癌发病年龄较早,肿瘤双侧、多中心发病率较高,应尽量行保留肾单位手术.     

Two cases of bilateral renal cell carcinoma in patients with Von Hipple-Lindau disease

Von Hipple-Lindau (VHL) disease is a rare familial cancer syndrome that is dominantly inherited and pre-disposes affected individuals to developing various tumors, including hemangioblastoma of the retina and central nervous system, and multicentric renal cell carcinoma. We report two cases of VHL disease with bilateral renal cell carcinoma. Case 1: A 53-year-old woman was referred to our hospital because of bilateral kidney tumor incidentally found. We performed left laparoscopic radical nephrectomy and laparoscopic nephrectomy, ex vivo excision and reconstruction, and autotransplantation for the right kidney. Case 2: A 43-year-old woman was referred to our hospital because of left kidney tumor incidentally found. Because the suspectious lesion in the right kidney was very small, we decided to follow it up with no treatment. We performed laparoscopic nephrectomy, ex vivo excision and reconstruction, and autotransplantation for left kidney.