Objectives: To report the results of conservative surgery for the treatment of hereditary renal cell carcinoma (RCC) and to define its place with respect to new less invasive treatment options.
Material and method: Over a period of 14 years, 30 women and 26 men were operated for one or several hereditary RCC, either by radical nephrectomy or nephron-sparing excision, via a lumbar or subcostal incision, when the diameter of at least one RCC was greater than 2.5 cm.
Results: 92 operations were performed: 62 local excisions and 30 nephrectomies. The TNM stage was: 75 pT1, 14 pT2, 3 pT3b; 4 N+; 3 M+. 26 patients (46%) were operated at least twice. Two or more RCC were resected in 43 cases (47%). In the case of nephron-sparing surgery, mean blood loss was 175 +/- 231.7 cc (range: 50-1300 cc); mean pedicle clamping time (97% of cases) was 32 +/- 10.4 min (range: 10-50 min); mean preoperative serum creatinine was 85 +/- 18 micromol/L (range: 52-150 micromol/L) and mean postoperative serum creatinine was 105 +/- 80 micromol/L (range: 59-576 micromol/L); the calculated tumour recurrence rate was 24% at 5 years and 80% at 8 years; overall 5-year survival was 100% and overall 10-year survival was 67%. The median follow-up was 55.9 months.
Conclusion: When hereditary RCCs are situated between 2.5 cm and 6 cm in diameter, nephron-sparing surgery is the reference treatment. Modern imaging and genetic screening should allow early detection of increasingly smaller RCCs. In the future, less invasive treatment options could replace surveillance for RCCs less than 2 cm and eventually reduce the indications for local excision.