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 w ith adult polycystic kidney disease. A 72-year-old white man with history o f adult polycystic kidney disease was first seen with gross hematuria. Cyst oscopy revealed bloody efflux from the right ureteral orifice. Computed tom ography (CT) revealed a mass in the right distal ureter. A right nephrouret erectomy showed multicystic changes. The distal ureter contained a mass sho wing morphologic features and immunohistochemical staining pattern consiste nt with small-cell carcinoma, intermediate cell type. Interphase cytogeneti cs studies using fluorescence in situ hybridization (FISH) showed abnormal chromosomal numbers for a number of chromosomes, including an interesting f inding of nulliploidy of chromosome 3. Aneuploid populations of tumor cells and a high S-phase fraction also were detected by flow cytometry. The sign ificance of these abnormalities of this rare tumor, with regard to their as sociation to the tumor's biologic behavior and their role in tumorigenesis, is discussed.