Abstract: Objective To explore the clinical characteristics of Von Hippel-Lindau(VHL)disease complicated with renal carcinoma, in order to find better methods of diagnosis and treatment. Methods Clinical data of 2 cases of VHL disease complicated with renal carcinoma were retrospectively analyzed and relevant literature was reviewed. Results One of the 2 patients had family history and underwent right radical nephrectomy. Pathological examination suggested clear-cell carcinoma. No recurrence was found in this patient after one-year follow-up. The other patient did not have family history. He also underwent right radical nephrectomy. Pathological examination suggested multilocular cystic renal cell carcinoma in the right kidney and pheochromocytoma in the right adrenal gland. Four years later, carcinoma was found in the left kidney and left adrenal gland. Pathological examination of biopsy tissue in the left kidney tumor indicated clear-cell carcinoma. He received sorafenib, a molecular targeted drug, and regular hemodialysis. Conclusion VHL disease is a rare cancer syndrome that involves multiple systems. The characteristics of renal cancer concurrent with VHL are different from sporadic one. Early diagnosis and individualized treatment can improve the quality of patients' life. New treatments should be further explored.

Key words: Von Hippel-Lindau disease, Renal tumor, Familial hereditary disease, Clinical characteristics