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ｖｏｎＨｉｐｐｅｌＬｉｎｄａｕ病及遗传性乳头状肾癌以双侧多发肾肿瘤为特点，易产生转移和新发肿瘤，平均寿命分别为４４岁和５２岁。为延长寿命、保留肾功能、提高生存质量、防止转移，可行保留肾实质的手术。作者分别对９６例ｖｏｎＨｉｐｐｅｌＬｉｎｄａｕ病及２
von Hippel-Lindau disease and hereditary papillary renal cell carcinoma are characterized by multiple bilateral renal tumors and are prone to metastasis and new-onset tumors with average life expectancy of 44 years and 52 years, respectively. In order to extend the life span, retain renal function, improve the quality of life, prevent the transfer, it is feasible to retain the renal parenchymal surgery. The authors were 96 cases of von Hip-pel Lindau disease and 2