RENAL-CELL CANCER WITH BOURNEVILLE PRINGLES DISEASE

We report a case of renal cell carcinoma in a 28-year-old woman with B ourneville-Pringle's disease. A review of the literature revealed 16 c ases of renal cell carcinoma associated with tuberous sclerosis. The i ncidence of angiomyolipoma among patients with Bourneville-Pringle's d isease is cited in the literature as 40-80%, but this is based solely on two early publications, while other publications suggest a consider ably higher incidence of renal cell carcinoma in Bourneville-Pringle's disease, as in other phacomatoses (Hippel-Lindau's disease). We there fore recommend screening for renal cell carcinoma in patients with Bou rneville-Pringle's disease.