Coexistence of nephrotic syndrome and neoplasm is rarely observed in children. We report the diagnostic and therapeutic problems of a 16-year-old female with nephrotic syndrome, ovarian tumor, and increased levels of tumor markers. She was suspected to have paraneoplastic nephrotic syndrome. After ovarian tumor resection, the nephrotic syndrome remission was not observed, while increased tumor marker levels were noted. The patient's final diagnosis was nephrotic syndrome in the course of primary mesangial proliferative glomerulonephritis. In conclusion, nephrotic syndrome in a patient with neoplasia might occur in the course of the primary and nonparaneoplastic glomerulopathy. Elevated serum tumor markers in patients with nephrotic syndrome might be nonspecific because of the stimulation of their production by peritoneal mesothelium, due to transudation to body cavities, that is, ascites.
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