Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience

Purpose: von Hippel-Lindau disease, hereditary papillary renal cell carcino ma, the Birt-Hogg-Dube syndrome and familial renal oncocytoma are familial renal tumor syndromes. These hereditary disorders are noteworthy for the de velopment of multiple bilateral renal tumors and the risk of new tumors thr oughout life. One management strategy is observation of solid renal tumors until reaching 3 cm., then performing parenchymal sparing surgery. We prese nt a 5-year update on our experience. Materials and Methods: From May 1988 to October 1998, 49 patients with here ditary renal cell carcinoma, including von Hippel-Lindau disease in 44, her editary papillary renal cell carcinoma in 4 and the Birt-Hogg-Dube syndrome in I, and I with familial renal oncocytoma underwent exploration to attemp t renal parenchymal sparing surgery. Patients were followed prospectively w ith periodic screening for recurrence, metastasis and loss of renal functio n. Median followup was 79.5 months (range 0.7 to 205). Results: A total of 50 patients underwent 71 operations resulting in unilat eral nephrectomy in 6, bilateral nephrectomy in I and partial nephrectomy i n 65, with 1 to 51 tumors removed from each kidney (mean 14.7). Mean patien t age was 39.5 years (range 18 to 70). Of the 65 (40%) partial nephrectomie s 26 were performed with cold renal ischemia. Mean blood loss was 2.9 +/- 0 .5 l. (range 0.15 to 23). Postoperative complications included renal atroph y in 3 patients. Mean preoperative serum creatinine was 1.05 +/- 0.03 mg./d l. (range 0.6 to 1.8), and postoperative creatinine was 1.06 +/- 0.04 mg./d l. (range 0.6 to 2.0). No patient who underwent renal parenchymal sparing s urgery required renal replacement therapy. Metastatic disease developed in 1 patient with a 4.5 cm. renal tumor. Conclusions: Parenchymal sparing surgery with a 3 cm. threshold in patients with hereditary renal cancer appears to be an effective therapeutic option to maximize renal function while minimizing the risk of metastatic disease .