[Kidney carcinoma in Bourneville-Pringle disease]

We report a case of renal cell carcinoma in a 28-year-old woman with Bourneville-Pringle's disease. A review of the literature revealed 16 cases of renal cell carcinoma associated with tuberous sclerosis. The incidence of angiomyolipoma among patients with Bourneville-Pringle's disease is cited in the literature as 40-80%, but this is based solely on two early publications, while other publications suggest a considerably higher incidence of renal cell carcinoma in Bourneville-Pringle's disease, as in other phacomatoses (Hippel-Lindau's disease). We therefore recommend screening for renal cell carcinoma in patients with Bourneville-Pringle's disease.