Pilot study of non‐contrast‐enhanced MRI vs. ultrasound in renal transplant recipients with acquired cystic kidney disease: a prospective intra‐individual comparison

The incidence of renal cell carcinoma (RCC) after kidney transplantation is 15‐fold increased. Acquired cystic kidney disease (ACKD) is one of the known risk factors. We performed a small pilot study to assess the role of non‐enhanced magnetic resonance imaging (MRI) as a tool for intensified screening in renal transplant recipients with ACKD. Renal ultrasound was used to assess the native kidneys of 215 renal transplant recipients. Thirty patients with 54 kidneys, fulfilling the criteria of ACKD, underwent non‐enhanced MRI at 1.5T using T2‐ and T1‐weighed as well as diffusion‐weighted sequences with a high spatial resolution. Among the 54 kidneys assessed by both methods, three RCCs were identified (6%). Of those, one RCC was detected by both imaging methods (33%), while two RCCs were diagnosed by MRI alone (67%). MRI identified an additional four proteinaceous or hemorrhagic cysts that did not fulfill the criteria for RCC but were classified as suspicious. All of these lesions were stable in size and appearance in follow‐up studies. In conclusion, non‐enhanced MRI was more sensitive than ultrasound in identifying RCCs and lesions suspicious for RCC and thus appears to be a useful secondary screening tool in patients with ACKD after renal transplantation.     

Renal cell carcinoma co-existent with other renal disease: clinico-pathological features in pre-dialysis patients and those receiving dialysis or renal transplantation.

BACKGROUND: Patients on chronic dialysis are prone to developing acquired cystic kidney disease (ACKD), which may lead to the development of renal cell carcinoma (RCC). The risk factors for the development of RCC so far have not been determined in pre-dialysis patients with co-existent renal disease. The aim of this study was to evaluate the clinico-pathological features of RCC in pre-dialysis patients with associated renal diseases or in those undergoing chronic dialysis and renal transplantation. METHODS: We studied 32 kidneys from 31 patients with RCC and associated renal diseases. Of those, 18 kidneys were from 17 patients not on renal replacement therapy (RRT) when diagnosed with RCC; 14 patients received dialysis or dialysis followed by renal transplantation. Several clinico-pathological features were analysed and compared between the two groups. RESULTS: Overall, there was a preponderance of males (75%); nephrosclerosis was the predominant co-existent disease (31%). The median intervals from renal disease to RCC in the dialysis and transplanted groups were significantly longer than in the pre-dialysis group (15.8+/-1.1 vs 2.4+/-0.7 years, P<0.0001). In contrast to pre-dialysis RCC, the dialysis and transplant RCC groups had greater frequency of ACKD (100 vs 28%, P<0.0001), papillary type RCC (43 vs 11%, P<0.05) and multifocal tumours (43 vs 5%, P<0.05). At the end of the study, 71% of dialysis and transplanted patients and 72% of pre-dialysis patients were alive. CONCLUSIONS: ACKD develops in dialysis patients, as it does in those with renal disease prior to RRT. The duration of renal disease, rather than the dialysis procedure itself, appears to be the main determinant of ACKD and RCC. The RCC occurring in patients with ACKD and prolonged RRT is more frequently of the papillary type and multifocal than the RCC occurring in patients with no or few acquired cysts and a short history of renal disease. Long-term outcomes did not differ between the two groups.     

Renal cell carcinoma in renal graft recipients and donors: incidence and consequence.

INTRODUCTION AND OBJECTIVES: Numerous studies have reported an increasing incidence of small renal cell carcinoma (RCC). De novo RCC in a renal allograft is a rare event and has special implications in renal transplant recipients. The objective of this study was to retrospectively evaluate the incidence of RCC in renal graft recipients and donors and to determine a procedure in cases with newly detected small renal tumors at the time of kidney preparation before transplantation. MATERIAL AND METHODS: We mailed a questionnaire to 38 German transplant clinics and received answers from 27 centers. A total of 10,997 renal graft recipients were included in the period of 1990-1998. RESULTS: In 30 kidneys (0.273%) RCC was detected at the time of preparation before transplantation. There were 23 male and 3 female donors. No bilateral RCC was described. The mean age of the donors with RCC was 50.9 years (range 37-72 years). The tumors had a mean size of 2.2 cm (range 0.4-6 cm). 67% of the patients had a renal tumor smaller than 20 mm. In 26/27 centers the decision to transplant relies on the result of the immediate section for microscopic examination. 16 patients (0.145%) developed RCC 3-12 years after renal transplantation (mean 7.4 years). The mean tumor size was 2.5 cm (range 2-2.8 cm). In 50% a grade 1 and in the other 50% a grade 2 carcinoma was found. CONCLUSIONS: Because of the RCC incidence in donor candidates we recommend an ultrasound screening of the native kidneys before renal explantation and an immediate preparation of the kidney surface especially in donors older than 45 years. In cases with small renal lesions we recommend an immediate section for microscopic examination before transplantation to prevent tumor implantation into an otherwise healthy patient. The frequency of RCCs after renal transplantation necessitates careful clinical and instrumental examinations in organ-transplanted recipients both before and at regular intervals after transplantation, including the patient's kidneys.     

长期血透患者获得性囊性肾病合并肾癌8例报告并文献复习

目的:学习长期血透患者获得性囊性肾病合并肾癌的筛查和诊治方法。方法:回顾性分析我院维持性血透获得性囊性肾病合并肾癌患者8例,均为B超和CT诊断为双肾多发性囊肿合并肾实质性占位,并行后腹腔镜下根治性肾切除术,术后维持规律性血透,并严密随访。结果:长期血透患者226例,获得性囊性肾病105例(46.5%),获得性囊性肾病合并肾癌8例(3.5%),在获得性囊性肾病中发生率为7.6%(8/105),其中男5例,女3例,年龄(58.6±16.4)岁,血透(12.2±6.9)年。8例患者(9次)行后腹腔镜下根治性肾切除术,手术均成功,出血(45.2±20.3)ml,手术时间(72.5±20.3)min,无严重手术并发症,术后病理3例为透明细胞癌和6例为乳头状癌。住院天数为(7.5±2.4)d。随访12～63个月,无瘤存活5例。结论:肾癌在获得性囊性肾病患者中发病率高,随着血透患者寿命的延长,血透3年后需重视和建立肾癌筛查机制,腹腔镜下根治性肾切除术安全有效、恢复快,并注重患者心脑血管疾病及糖尿病等并发症的积极治疗,有助于进一步延长血透患者寿命。     

Transplant of kidneys with small renal cell carcinoma in incompatible, heavily immunosuppressed recipients

Renal cell carcinoma (RCC) is considered a contraindication for transplant. However, an increasing number of cases of transplant kidneys with RCC have been reported with encouraging results. We present our experience of two cases of transplanting kidneys with small RCCs. Donors and recipients were aware of the presence and possible consequences of RCC in the transplanted kidney before transplantation. Cases were discussed in the multidisciplinary team meetings. Regular, 6-12 monthly follow-up of donors and recipients was carried out with ultrasonography and/or computed tomography to detect recurrence of RCC or new tumours in the recipients' transplant kidneys or the donors' native kidneys. The outcome was recorded. There were no suspicious masses in the any of the kidneys during the follow-up period. The transplant kidneys are functioning.     

The long-term effect of simultaneous heart and kidney transplantation on native renal function.

BACKGROUND: It is unclear whether patients with heart failure and renal insufficiency should receive a simultaneous heart and kidney transplant or whether a single heart transplantation is sufficient to restore native renal function. METHODS: We analyzed the renal plasma flow and glomerular filtration of the native and transplant kidneys in eight patients long term after simultaneous heart and kidney transplantation using a dynamic MAG3 radioisotope scan and serum creatinine determinations. All subjects had been hemodialysis dependent before transplantation. Seven patients suffered from an intrinsic renal disease that were diabetic nephropathy in three cases, small fibrotic kidneys of undetermined origin in two cases, one lupus nephritis, and cyclosporine nephrotoxicity in one patient who had a previous heart transplant. In one patient renal insufficiency was considered to be solely due to renal hypoperfusion because no intrinsic renal disease could be detected. RESULTS: All patients were on cyclosporine-based triple immunosuppression, transplanted for 4 to 10 years, exhibited cardiac ejection fractions of more than 50% and had normal serum creatinine values. Radioisotopic scan showed no function of the native kidneys in all seven patients with intrinsic renal disease but exhibited normal function of the native kidneys as well as the renal transplant in the patient without intrinsic kidney disease before transplantation. CONCLUSIONS: These data suggest that a simultaneous heart and kidney transplantation is necessary in patients with cardiomyopathy and renal insufficiency due to primary kidney disease, but not in those with hemodynamically mediated renal failure, even if an immunosuppressive regimen with calcineurin inhibitors is used.     

获得性肾囊性疾病合并肾癌11例临床分析

目的 探讨获得性肾囊性疾病(ACKD)合并肾癌的诊断治疗策略.方法 回顾性分析11例终末期肾衰竭、获得性肾囊肿合并肾癌患者的临床资料.男8例,女3例.平均年龄55(37～68)岁.行血液透析至发现肾脏病变时间平均为4.8(2.8～7.4)年.结果 11例均行肾癌根治术.术后病理报告:透明细胞癌3例,乳头状癌6例,嫌色细胞癌1例,乳头状腺瘤1例.病理分期T1a9例,T1b 2例.11例术后随访平均55(17～83)个月.1例术后24个月发现肺转移;1例单侧发病者术后22个月对侧复发,行肾癌根治术;1例死于心血管疾病;1例随访19个月后失访;无瘤生存7例.结论 ACKD与肾癌有较高的相关性.终末期肾衰竭患者透析前氮质血症时间较长或透析时间＞3年者,应排除ACKD.超声及CT检查对早期诊断存在价值.除关注ACKD恶性变倾向外,对长期肾衰竭患者的其他并发症如心脑血管疾病、糖尿病等也应足够重视并行积极治疗.

Abstract：

Objective To discuss the diagnosis and treatment of acquired cystic kidney disease complicated by kidney cancer. Methods Clinical data of 11 patients with acquired cystic kidney disease complicated by kidney cancer were analyzed retrospectively. Eight patients were male and three were female. The mean age was 55 years old (range 37 to 68). The time of hemodialysis ranged from 2.8 to 7. 4 years, mean 4. 8 years. Results Follow-up ranged from 17- 83 months, mean 55 months. One patient died of cardiovascular disease. Lung metastasis was detected in one patient two years after surgery. Seven patients survived free of tumor recurrence and there was no follow-up on one patient. Conclusions Increased incidence of cancer was observed in patients with end-stage renal disease who have undergone long-term dialysis. In particular, renal cell carcinoma (RCC) showed an excess incidence in ACKD patients. RCC showed an increased prevalence compared with the general population. Patients with predialysis azotemia or a dialysis duration of longer than 3 years should be screened for ACKD. Sonegraphy or CT scanning are useful for early diagnosis of ACKD. We should pay close attention to complications, including ACKD malignant tendency, in patients who have been taking long-term dialysis and positive therapy.     

Renal cell carcinoma of native kidney in renal transplant recipients

OBJECTIVE: To evaluate the prevalence, prognosis and possible risk factors of renal cell carcinoma (RCC) of the native kidney in renal transplant recipients. PATIENTS AND METHODS: We retrospectively re-examined the follow-up data of 373 consecutive renal transplant recipients at our institution between August 1993 and September 2004. We collected the data of all de novo RCC of the native kidney in the current analysis. RESULTS: Of the 373 patients examined, 12 tumours of the native kidney were diagnosed in 10 individuals. The mean ages at transplantation and diagnosis were 33 and 45.8 years, respectively. Thirteen malignancies were discovered fortuitously. Among the renal ultrasonograms there were two false-negative results. The mean tumour size was 21 mm. Nephrectomy was performed in all cases. Among the 12 kidney malignancies, there were five conventional RCCs and seven papillary RCCs. Half of all tumours were Furhman Grade 3 lesions, and pT1aN0M0 tumours also accounted for all malignancies in the current cohort. One of the 10 patients died, from progression of metastases 6 years after diagnosis. One patient had a local recurrence 2 years after diagnosis. The other eight patients were alive with no evidence of disease at the time of the current report. No significant relationship was detected between RCC occurrence and clinical patient characteristics. CONCLUSIONS: There appears to be a greater risk of RCC of the native kidney in patients with end-stage renal disease. The present results suggest that an annual examination of the native kidney before and after renal transplantation is essential.     

The Role of Microscopic Hematuria in the Evaluation of Urologic Malignancy in Renal Transplant Recipients

Urologic malignancy is a relatively uncommon but serious complication following kidney transplantation. The reported prevalence of renal cell carcinoma (RCC) of the native kidneys is 4.4% and of bladder malignancy is 2.6%. However, presently there are no universal guidelines for prospective screening of urologic malignancies after kidney transplantation. We routinely monitored all renal transplant recipients for microscopic hematuria and persistent hematuria (>3 separate occasions) results in imaging studies (ultrasound or computed tomography scan) of both native kidneys and the allograft. Cystoscopy is performed if imaging studies are negative. This retrospective study identified a total of 18 urologic malignancies among the study cohort, which consisted of 539 patients with an incidence of 3.3% (12 cases of RCC of native kidneys [10/12 had hematuria], and six cases of bladder and ureteral malignancies [6/6 had hematuria]). There were no significant differences between cyclosporine- and tacrolimus-based immunosuppression (IS). Among RCC recipients, two lost the allograft from chronic allograft nephropathy and one patient died unrelated to malignancy. Among patients with bladder and ureteral malignancies, two lost the graft possibly from IS reduction and one had BK virus nephropathy prior to diagnosis of bladder carcinoma. In conclusion, screening transplant recipients routinely for persistent microscopic hematuria may identify urologic malignancies in renal transplant recipients.     

Acquired cystic kidney disease: rapid progression from small to enlarged kidneys simulating adult polycystic kidney disease

A 57-year-old man on chronic hemodialysis presented marked bilateral renal enlargement due to acquired cystic kidney disease (ACKD). He had been on hemodialysis for less than 3 years only (14 months prior to receiving a functional renal transplant which lasted 8 years, followed by 18 additional months of dialysis), before the diagnosis of ACKD was made following an episode of flank pain with gross hematuria. The marked changes in kidney appearance during this 11-year period were documented by serial ultrasound examination showing the kidneys to be of near-normal size before the start of dialysis (> or =10 cm in 1986), then shrunken and contracted 5 years later while having a functioning renal transplant (<5 cm in 1991), and markedly enlarged reaching the size of adult polycystic kidney disease after returning to dialysis (>13 cm in 1997). Since the risk of ACKD increases with duration of dialysis, we sought additional predisposing factors in this unusual case and found that 2 years after renal transplantation, the patient was diagnosed with breast cancer for which he was treated with surgical excision and tamoxifen. Based on ultrasound evidence that the tamoxifen treatment preceeded the appearance of the renal cystic changes, we wonder whether this drug may have played a role in the rapid development of ACKD.     

A lucky fall? Case report

Renal cell carcinomas (RCCs) account for 3% of all solid neoplasms, with an increased incidence after renal transplantation. In transplant recipients, RCCs predominantly occur in the patient's native kidneys. Herein is reported a case of a localized RCC of recipient origin that developed in the donor allograft and was detected 8 years after renal transplantation. Treatment with high-intensity focussed ultrasound followed by partial nephrectomy was successful, averting the need for dialysis therapy.     

Indication, surgical technique and outcome of orthotopic renal transplantation

PURPOSE: We review the indication, surgical technique and outcome of orthotopic renal transplantation. MATERIALS AND METHODS: The medical records of 1,000 patients who underwent renal transplantation at our institution between August 24, 1993 and August 1, 2000, as well as orthotopic renal transplantation were reviewed. RESULTS: Orthotopic renal transplantation was performed in 4 males and 1 female with severe iliac atherosclerosis or retained bilateral iliac fossa kidney transplant. Mean patient age was 56 years. There were 2 patients who received kidneys from living related donors, and 3 underwent cadaveric renal transplantation. Left orthotopic renal transplantation was successful in 4 cases, and 1 was converted to iliac fossa renal transplant because of a pulseless splenic artery and renal artery thrombosis after native renal endarterectomy. Orthotopic renal revascularization was done with splenic artery in 2, native renal artery in 2 and left renal vein in all 4 patients. Urinary tract reconstruction was performed with stented (2) or nonstented (2) ureteroureterostomy. Antibody induction, purine antagonists, calcineurin inhibitors and glucocorticoids were used for immunosuppression. Mean preoperative and 1-month postoperative serum creatinine was 7.9 and 1.3 mg./dl., respectively. Patient and graft survival was 100% during followup, which ranged from 6 months to 5 years. CONCLUSIONS: Despite the technical challenges, orthotopic renal transplantation in patients with unsuitable pelvic vessels can result in excellent patient and graft survival.     

Successful Surgical Treatment of Renal Cell Carcinoma in a Transplanted Kidney from a Cadaveric Donor: Report of a Case

Posttransplant renal cell carcinoma (RCC) usually arises in the native kidneys of renal transplant recipients rather than in the transplanted kidney. This report describes a case of RCC that developed in the transplanted cadaveric kidney in a 37-year-old male recipient 9 months after transplantation. An en bloc radical transplant nephrectomy was performed, and he has subsequently remained stable on hemodialysis for 3 years without any sign of recurrence. Received: March 27, 2000 / Accepted: September 26, 2000     

Organ donors with small renal cancer: report of 3 cases

Transmission of donor malignancies has been reported since the early days of clinical transplantation. Up to 1995, the Transplant Tumor Registry created by Penn included 155 cadaver and 29 living donor affected by tumors. The most common, excluding brain tumor, was renal cell carcinoma (RCC). RCC represents 2% of adult cancers, an incidence that increases with advancing age. The expansion of the criteria that define a suitable organ donor has as a consequence included donors that are older than in the past. Small RCCs are found during renal recovery from a cadaveric donor in ∼1% of cases. The use of such donors is a matter of debate; it has been suggested that donor kidneys with small RCC Fuhrman grade I/II may be transplanted after appropriate surgical excision. We report our experience with 3 donors with clear cell RCC: 2 contralateral kidneys were transplanted in 2 recipient and a third recipient received an affected kidney after a wide tumor excision. All of the patients we alive and free from recurrence at 14-48 months (mean 35 mo). In the third case, immunosuppression was achieved with a mammalian target of rapamycin inhibitor, which is currently used not only as an immunosuppressant to prevent rejection, but also as treatment for renal cancer. Our data confirmed that donors with small renal tumors may be used, because the risk of tumor recurrence is small and the benefits of a kidney transplantation are great.     

Features and outcomes of renal cell carcinoma of native kidneys in renal transplant recipients

Study Type – Therapy (case series)
Level of Evidence 4

OBJECTIVE

To outline the features and outcomes of renal cell carcinoma (RCC) in native kidneys of renal transplant recipients, who are at increased risk of developing this disease.

PATIENTS AND METHODS

We retrospectively studied the clinicopathological features and survival of 28 surgically treated RCCs, which developed in 24 renal transplant recipients. Features and outcomes were compared with 671 patients with RCC who had no renal transplant.

RESULTS

The median interval between renal transplantation and the occurrence of RCC was 5.6 years. Acquired cystic kidney disease was present in 83% of the transplanted patients. Compared with the patients with RCC and no renal transplant, RCCs of native kidneys in transplant recipients were more frequently incidental findings (92% vs 77%, P = 0.092), multifocal (39% vs 15%, P < 0.001), bilateral (17% vs 4%, P = 0.006), had lower T stages (P = 0.040), were smaller (P = 0.027), of lower grades (P = 0.010), were more frequently papillary (43% vs 19%, P = 0.019) and occurred at a significantly younger age (P = 0.022). After a median follow‐up of 6.7 years, eight renal transplant recipients had died (33%), but only two deaths were due to RCC. Survival with metastatic RCC was only 4 months, if a full resection of all metastatic sites was not achieved. In multivariate analysis the presence of a renal transplant had no effect on survival.

CONCLUSIONS

Most RCCs in renal transplant recipients are incidental low‐stage, low‐grade tumours with a favourable prognosis. The outstanding pathological findings are bilateral occurrence, papillary subtype and multifocality. Prognosis of metastatic RCC is poor but might be favourable if all metastases are resected. Screening for early detection of asymptomatic RCC is advocated.     

Renal cell carcinoma suspected at time of organ donation 2008‐2016: A report of the OPTN ad hoc Disease Transmission Advisory Committee Registry

All 179 reports to the OPTN of potential renal cell carcinoma (RCC) transmission from 1/1/2008 through 12/31/2016 were reviewed. Cases were divided into those with donor tumor known or suspected at time of transplant (N = 147 donors), and those in which tumor was initially found after transplant (N = 32). We sought to understand the risk of transplanting either the affected kidney, the contralateral kidney or non‐renal organs from donors with a suspected/confirmed unilateral RCC. In the case of RCC found prior to transplant, transplantation of 21 kidneys following excision of tumor, 47 contralateral kidneys and 198 non‐renal organs was performed. No cases of RCC transmission were documented in this population. An additional six cases of live donor kidney transplantation involving resection of RCC were reported, also without transmission. Six of 9 other recipients in whom the diagnosis of RCC became available after implantation underwent allograft nephrectomy and 3 received tumor resection. No recurrent RCC was documented. Given the low rate of transmission and available treatment options, consideration should be given to judicious use of organs from donors with small solitary RCC.     

Renal Cell Carcinoma of Native Kidney After Renal Transplantation: Clinical Relevance of Early Detection

Background

Life expectancy after transplantation has improved, and cancer may soon be the leading cause of late death after transplantation. The guidelines of the American and European societies of nephrology and urology have not yet established the optimal frequency for screening for renal cell carcinoma (RCC) of native kidneys in patients who have undergone renal transplantation.

Objective

To evaluate the prevalence, prognosis, and risk factors of RCC in a series of patients followed up for 16 years in our transplantation unit.

Materials and Methods

Our study is a follow-up observational cohort study conducted in 694 consecutive renal transplant recipients admitted to our institution from July 1991 through July 2007. At our institution, ultrasound studies of the native kidneys were performed every 6 months after renal transplantation.

Results

In the patient cohort studied, 10 patients developed a renal tumor (1.6% incidence). Three patients died of causes other than recurrence of RCC. Seven patients are alive with no evidence of RCC recurrence or metastatic disease after a mean (range) follow-up of 41 (12–96) months. Acquired cystic kidney disease and dialysis duration were positively associated with development of RCC.

Conclusions

The incidence of RCC in the literature varies between 0.3% and 4.8%. The variability depends on the timing of follow-up, with a higher incidence in prospective studies with strict follow-up. We advise ultrasound studies performed by specialized physicians every 6 months after transplantation. More detailed guidelines designed by the major international transplantation societies are necessary.     

Severity of acquired renal cysts in native kidneys and renal allograft with long-standing poor function

To identify factors related to the development of uremic acquired cystic disease of the kidney, native and grafted kidneys were examined in four men and three women after kidney transplantation. The incidence and severity of cystic transformation of native kidneys and grafts were compared by plain computed tomographic scans. In a uremic environment (serum creatinine level of greater than or equal to 265 mumol/L [3 mg/dL] for an average of 5.0 years; range, 2.8 to 8.2 years), acquired renal cysts were formed in both the native kidneys and the graft in three of the male and one of the female patients. Cysts were extensive in the native kidneys but relatively infrequent in the grafts in three of the men. One male subject was found to have acquired cysts only in the native kidney. Acquired renal cysts developed even in grafts undergoing chronic rejection, and increased numbers were found in native kidneys that were in uremic conditions for long periods, both before and after renal transplantation. These results suggest that the duration of uremia is the most important factor in the development of acquired renal cysts.     

Does growth hormone treatment affect the risk of post-transplant renal cancer?

According to the analysis of the Collaborative Transplant Study (CTS), the incidence of renal carcinoma in patients with renal transplantation as well as with heart transplantation is significantly increased at any given patient age. The cumulative incidence 10 years after kidney transplantation is 185 per 100,000 patients in children below the age of 19 years at the time of transplantation. Age and immunosuppressive treatment seem to be the major risk factors. The majority of cancers develop within the native kidneys. Chronic transplant nephropathy and accelerated senescence may be further risk factors for the development of cancer within a kidney transplant. Growth hormone (GH) treatment could not be identified as an additional risk factor.     

Outcome of patients with tuberous sclerosis after renal transplantation

The fate of tuberous sclerosis (TS) patients after renal transplantation (RT) for end-stage renal failure remains to be defined. We report three patients with a posttransplantation follow-up averaging 54 months and review 6 previously published cases. Three women, aged 27-46 years, received a cadaver kidney 26-67 months after starting dialysis. None had mental retardation, 2 had suffered from seizures during infancy and 2 had intracranial calcification; neurological involvement was equally mild in the 6 reported patients. Currently, 16-84 months after RT, our 3 patients are fully rehabilitated with a well-functioning graft (serum creatinine 1.2-1.7 mg/dl). Results of RT are also satisfactory in the 4 other reported cases for whom a follow-up is available, except for 1 death unrelated to the initial disease. Neurologic disorders did not progress. Renal cell carcinoma was discovered in one removed kidney, and cells suggestive of malignant transformation in another case. No metastases were discovered up to 4 years later. No neoplastic transformation was observed up to 7 years after RT in the 3 patients who retained their native kidneys. TS patients with end-stage renal failure are good candidates for RT. The probably small risk of neoplastic transformation of native kidneys warrants a close monitoring by CT scan of the few patients who have not undergone bilateral nephrectomy.