The surgical management of hereditary multifocal renal carcinoma

A family was described recently in which renal carcinoma developed in 10 members in 3 consecutive generations. The cancer tended to develop in patients at an early age, and to occur in both kidneys and at multiple sites more frequently than in nonfamilial renal cancer. The finding of a balanced reciprocal translocation between chromosomes 3 and 8 in affected family members suggest that an inherited genetic defect predisposes to the development of this cancer in middle-aged patients and offers a marker for screening individuals at risk. We herein discuss the surgical management of 4 family members, the proband and 3 relatives, who were discovered on screening to have bilateral, multifocal renal cancer. Certain compromises in the principles of radical tumor surgery have proved effective in these 4 patients. Partial nephrectomy has resulted in total extirpation of the bilateral tumors with survivals of 2 to 4 years without need for chronic dialysis. The sequential order of surgical procedures is important since partial nephrectomy of the least affected side first allows a total nephrectomy on the more affected side to be done later, when necessary, without transient renal failure.