Renal cell carcinoma in children with diffuse cystic hyperplasia of the kidneys

被引:8
作者
Henske, EP
Thorner, P
Patterson, K
Zhuang, ZP
Bernstein, J
机构
[1] Fox Chase Canc Ctr, Dept Med Oncol, Philadelphia, PA 19111 USA
[2] Hosp Sick Children, Div Pathol, Dept Pediat Lab Med, Toronto, ON M5G 1X8, Canada
[3] Childrens Hosp & Med Ctr, Dept Pathol, Seattle, WA 98105 USA
[4] NCI, Dept Pathol, Bethesda, MD 20892 USA
[5] William Beaumont Res Inst, Royal Oak, MI 48073 USA
关键词
renal cell carcinoma; polycystic kidney; tuberous sclerosis; von Hippel-Lindau disease;
D O I
10.1007/s100249900123
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
We report the clinical, pathologic, and genetic features of renal malignancy in two children with diffuse cystic hyperplasia. Both presented with massive bilateral nephromegaly. Neither had a family history or clinical findings suggestive of tuberous sclerosis or von Hippel-Lindau disease. The kidneys of both children were extensively replaced by tubulocystic hyperplasia with large eosinophilic epithelial cells. The masses of hyperplastic tissue were nodular, compressing remnants of uninvolved renal parenchyma. Tubulopapillary carcinoma was present in both children, one of whom had bilateral multicentric carcinoma. No loss of heterozygosity was detected in the tumors at the TSC1, TSC2, or VHL gene regions, and no alterations in the VHL gene were detected using single-strand conformation polymorphism analysis. These cases of bilateral renal enlargement with diffuse cystic hyperplasia appear to represent a new clinical syndrome that may warrant bilateral nephrectomy because of the risk of malignancy.
引用
收藏
页码:270 / 274
页数:5
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