[Clinical analysis in diagnosis and treatment of 11 patients with hereditary renal cell carcinoma].

OBJECTIVE

To evaluate the diagnosis and treatment of hereditary renal cell carcinoma.

METHODS

Clinical data of 11 patients with hereditary renal cell carcinoma were analyzed retrospectively. Eight patients were male and 3 were female, age ranged from 32 to 67 (mean: age 48 years). Four cases were bilateral renal cell carcinoma, and 4 were multiple renal cell carcinoma. Two cases were diagnosed as Von Hippel-Lindau syndrome, 6 as familial clear cell renal cell cancer, and 3 as hereditary papillary renal carcinoma.

RESULTS

Ten patients performed nephron-sparing surgery and/or radical nephrectomy and 1 had no operation. The patients were followed up from 12 to 114 months. Tumor recurrence was observed in 4 patients, 1 patient died of tumor metastasis, and 2 died of other causes. Four patients survived free of tumor.

CONCLUSIONS

Hereditary renal carcinoma appears in the youth, and it is predominantly multiple and bilateral. Nephron-sparing surgery is the standard method of treatment for the patients.