Urothelial cancer in patients with Endemic Balkan Nephropathy (EN) after renal transplantation.

GOAL: Analysis of the incidence of urothelial cancer and outcome of treatment in patients with Endemic Balkan Nephropathy (EN) after renal transplantation. METHODS: From January 1985 until October 2006, 550 kidney transplantations (389 cadaveric) and 5 combined kidney and pancreas transplantations were performed in University Hospital Center Rijeka. In only 6 (1.1%) of 555 transplant recipients, EN was diagnosed as the original kidney disease, based on medical history, clinical findings, and laboratory results, but without pathohistologic verification. All patients with EN received the first renal transplant from a cadaver. Patients' mean age at transplantation was 50.3 +/-15.9 yrs, five patients (83.3%) were male. The incidence of malignant tumors in all 555 transplant recipients was analyzed, with an emphasis on the incidence of urothelial cancer and outcome of treatment in the group of patients with EN. RESULTS: During posttransplant follow-up period, malignancy was diagnosed in 27 (4.9%) out of 555 transplant recipients. Skin cancer was diagnosed in 7 patients (1.3%), followed by cancer of the urinary tract in 6 patients (1.1%) and breast cancer in 3 patients (0.5%). In 3 of 6 patients with EN, urothelial cancer was diagnosed, resulting in the death in two patients. In the third patient, urothelial cancer showed a high affinity for recurrence, and besides the strong reduction of immunosuppressive therapy, repeated surgical treatment was needed. CONCLUSIONS: Patients with EN show a high incidence of urothelial cancer after renal transplantation. A thorough nephro-urological evaluation is needed before transplantation, and a careful follow-up is required afterward to ensure an early diagnosis of malignancy. Preventive nephroureterectomy is recommended.

Renal transplantation in patients with Balkan endemic nephropathy.

BACKGROUND: Balkan endemic nephropathy (BEN) is a chronic tubulointerstitial disease prevalent in Croatia, Romania, Bulgaria, Bosnia and Herzegovina, and Serbia. In addition to renal disease, an increased incidence of upper urothelial carcinomas (UUCs) has been observed in the foci of BEN. Carcinoma may occur alone or in combination with BEN. Immunosuppression is associated with an increased risk for development of different malignancies. There are no data in the literature about the outcome of patients with BEN after transplantation. METHODS: We performed a retrospective evaluation of the database and review of the charts and pathology reports of 601 renal transplant recipients treated at our institution. RESULTS: From January 1995 to December 2004, kidney transplantations were performed in nine patients with BEN. One-year graft survival was 100%. A man, who was transplanted in 1997 died 2 years after transplantation with a functioning graft due to disseminated cancer from the pelvis of his own kidney. A female patient developed UCC 2 years after transplantation. They were both treated with a bolus of methylprednisolone before transplantation, because of four HLA-mismatches. A male patient developed UCC in the native and transplanted kidneys. He underwent a native nephroureterectomy with partial nephroureterectomy of transplanted kidney. His graft function was preserved with decreased immunosuppression. Three years later a urinary bladder carcinoma was discovered on a regularly performed multislice computed tomography. One patient developed a skin malignancy. Other patients have had uneventful posttransplantation courses with excellent graft function. Thus, 33.3% of patients with BEN developed UUC, compared with a 0.67% prevalence of urinary tract tumors among transplanted patients with other causes of end-stage renal disease. CONCLUSION: Patients with BEN are at increased risk for the development of UCC after transplantation. Regular screening for early detection of malignancy is mandatory. Longer follow-up and results from other transplant centers are needed to further investigate the relationship between BEN and UCC after renal transplantation.

Conservative surgical treatment of low-grade urothelial carcinoma in the renal allograft recipient: a case report.

Development of urothelial carcinoma in a renal allograft is rare. We report the case of 52-year-old male patient who developed chronic renal failure secondary to Balkan endemic nephropathy and underwent renal allotransplantation. The patient who developed low-grade pTa urothelial carcinoma in the left contracted kidney at 3 years after transplantation and underwent nephroureterectomy. Three years later, the same neoplastic process was observed in the renal allograft. Preoperative estimation for allograft tumor recurrence and progression included percutaneous tumor biopsy followed by cytopathological, histological, and cytogenetic analysis. Cytopathology revealed well-differentiated urothelial tumor cells. Histopathologic analysis showed low-grade urothelial carcinoma. Cytogenetic examination demonstrated that the tumor originated from the recipient suggesting a low malignant potential of carcinoma. Based on these findings, we decided to perform a right-side nephroureterectomy and graft-sparing procedure, which resulted in preservation of allograft function. In this report we discussed the prognostic factors, which are the basis for rational therapeutic approaches in these patients.

[Urothelial tumors versus "endemic" nephropathy - myth or reality?]. / Urothelial tumors versus "endemic" nephropathy - myth or reality?

Malignant tumors of the renal pelvis account for over 78 per cent of all malignant tumors of the kidney, and less than 1 per cent of all urogenital neoplasms. At the time of diagnosing, almost one third of these patients present with tumor of the ipsilateral ureter or bladder, and 40-50 per cent have ureteral tumor located elsewhere (D. Crawford, S. Das, 1990). After World War Two, the frequency of publications on cases of primary tumors of the pelvis show a noticeable increase, e.g. in Yugoslavia and Bulgaria the ratio of parenchymatous renal tumors to those of the renal pelvis is conspicuously altered. S. Petcovic (1970) and S. Lambrev (1972) attribute this fact to the existence of endemic "nephropathy" foci. It is the purpose of this work to analyze twenty-nine patients presenting carcinoma of the upper urinary ways, studied in the Chair of Urology in the period 1991 through 1997. Of them only four come from "endemic" regions. Over the period 1972-1975, fifty-nine patients with the same condition undergo treatment in the aforementioned Chair. It is worth noting that patients from the so-called "endemic" regions lack the typical signs of "endemic" nephropathy. The assumption is warranted that "endemic" nephropathy is a still not well enough clarified nosological entity, bearing resemblance to contamination with radioactive elements with a "boom" during the half-life period gradually subsiding.