Renal cell carcinomas in children and young adults: increased incidence of papillary architecture and unique subtypes.

摘要: Renal cell carcinomas in children and young adults are rare, the pathologic features of these tumors have not been well described. We reviewed 24 renal ages 6 to 29 years, 14 whom were younger than 18 years age. Fourteen female. In 19 (79%) cases, tumor met histologic criteria for papillary carcinoma, with at least 50% architecture. Four remaining five cases typical clear patients known von Hippel Lindau syndrome, one case was chromophobe type. tumors, calcifications, high nuclear grade, extracapsular extension (American Joint Commission on Cancer stage T3), lymph node metastases common. Among four distinct patterns could be identified. Collecting duct-like (two cases) involved large collecting ducts, multifocal predominantly papillary, had focal tubular solid areas. These reactive epithelial membrane antigen (EMA) keratins, including CK7, but negative Ulex europeaus molecular weight keratin 34BE12. Voluminous (four composed cells extremely voluminous cytoplasm and, although areas that also resembled tumors. keratins AE1/AE3 otherwise all other EMA, U. europeaus. One showed an X;7 translocation. Adult type (12 adults. EMA This last trisomies chromosomes 7, 16, 17, 20. The final neuroendocrinelike multifocal, organoid, nests small a pattern. Three 13 alive disease follow-up, three additional died disease, within 2 years. Progression highly associated involvement time resection. conclude clinicopathologic differ from those arising older characteristically high-grade, high-stage, numerous several subtypes can identified based histologic, immunohistochemical, cytogenetic features. Some appear unique this age group.