VONHIPPEL-LINDAU DISEASE AND RENAL-CELL CARCINOMA IN A 16-YEAR-OLD BOY

被引：29
作者：
KEELER, LL
KLAUBER, GT
机构：
[1] Department of Pediatric Urology, New England Medical Center Hospitals, Tufts University, Boston, MA
来源：
JOURNAL OF UROLOGY | 1992年 / 147卷 / 06期
关键词：
VONHIPPEL-LINDAU DISEASE; ANGIOMATOSIS; CARCINOMA; RENAL CELL; PEDIATRICS;
D O I：
10.1016/S0022-5347(17)37636-X
中图分类号：
R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];
学科分类号：
1002 ; 100201 ;
摘要：
Von Hippel-Lindau disease is a rare autosomal dominant disorder. Kidney lesions occur in the majority of cases, with renal cell carcinoma noted in 40% and renal cysts in 60%. Renal cell carcinoma in von Hippel-Lindau disease is usually bilateral and occurs at an earlier age than in patients with sporadic renal cell carcinoma. We report on a 16-year-old boy who, to our knowledge, is the youngest patient to present with von Hippel-Lindau disease and renal cell carcinoma. Controversy currently exists regarding the nature of renal cysts in von Hippel-Lindau disease and the optimal therapeutic approach (that is radical versus parenchymal sparing surgery). We review the histology of renal cysts and carcinoma, and discuss the rationale for selecting parenchymal sparing surgery.
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收藏
页码：1588 / 1591
页数：4
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