Small Cell Carcinoma of the Ureter Arising in an Adult Polycystic Kidney Disease: A Case Report

We report a case of primary small-cell carcinoma of the ureter associated with adult polycystic kidney disease. A 72-year-old white man with history of adult polycystic kidney disease was first seen with gross hematuria. Cystoscopy revealed bloody efflux from the right ureteral orifice. Computed tomography (CT) revealed a mass in the right distal ureter. A right nephroureterectomy showed multicystic changes. The distal ureter contained a mass showing morphologic features and immunohistochemical staining pattern consistent with small-cell carcinoma, intermediate cell type. Interphase cytogenetics studies using fluorescence in situ hybridization (FISH) showed abnormal chromosomal numbers for a number of chromosomes, including an interesting finding of nulliploidy of chromosome 3. Aneuploid populations of tumor cells and a high S-phase fraction also were detected by flow cytometry. The significance of these abnormalities of this rare tumor, with regard to their association to the tumor's biologic behavior and their role in tumorigenesis, is discussed.