Abstract：Objective: To explore the surgical procedures for Von Hippel-Lindau (VHL) syndrome accompanied with renal cell carcinoma (RCC). Methods: From March 2009 to August 2013, 9 cases of VHL syndrome accompanied with RCC were subjected to treatments in PLA General Hospital. Family history, clinical manifestations, characteristics of RCC and surgical treatments were retrospectively studied. Results: Most of the patients were 20-40 year old, and 4 out of 9 cases had significant family history of central nervous system (CNS) hemangioblastoma. However, the rest had CNS hemangioblastoma without significant family history. Besides RCC, this kind of syndrome included pancreatic tumor or pancreatic cyst, adrenal pheochromocytoma, etc. All of RCC were bilateral or multi-focus clear cell RCC. The grade and stage of these RCC were low. Nephron sparing surgery was common surgical treatment for these groups, including laparoscopic and open approaches. Nine patients with RCC underwent 10 operations, with a mean operation time of 142.5 min, mean blood loss of 163.3 mL and mean hospital stay of 7.2 days. Conclusions: VHL syndrome accompanied with RCC is an inherited disease with multiple lesions. Surgical operation is the first choice for treatment of renal tumors, and laparoscopic nephron sparing operation is safe, reasonable and effective treatment for this kind of syndrome.
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