Jc. Herring et al., Parenchymal sparing surgery in patients with hereditary renal cell carcinoma: 10-year experience, J UROL, 165(3), 2001, pp. 777-781
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
.