The clinical and pathological manifestations of renal tumors in von Hippel-Lindau disease.

The protean nature of renal lesions in von Hippel-Lindau disease has made surgical management difficult. To develop a sensible surgical approach the pathological and clinical manifestations of renal involvement in von Hippel-Lindau disease patients were defined. A total of 87 lesions in 9 kidneys from 6 patients with von Hippel-Lindau disease was examined for size, presence of renal cell carcinoma, cystic or solid qualities and local invasion. There was no correlation between the size of the lesion and renal cell carcinoma. All 44 benign lesions were cystic, while 35% (15 of 43) of malignant lesions were cystic and 65% (28 of 43) were solid. All 9 locally invasive tumors were solid. The distribution of lesions in our patients was compared to the findings in 138 cases we collected from the literature. The 3 patterns of clinical presentation recognized were solitary, multiple and diffuse. We recommended that all lesions, cystic or solid, be excised at the time of nephron sparing surgery, with a wider margin for solid lesions, and that nephron sparing surgery should be reserved for patients with cystic and small solid lesions, with radical nephrectomy being preferred for patients with diffuse disease.