von Hippel—Lindau综合征外科治疗

Surgical management of yon Hippel-Lindau disease（report of 4 cases）

目的探讨vonHippel—Lindau（VHL）综合征的外科治疗方法。方法VHL综合征患者4例。例1，男，56岁。主诉乏力、心悸2d。空腹血糖2．37mmol／L。CT检查示左肾上、下极3个肿块，直径分别为8．0、7．0、4．0cm。10年前行脑血管母细胞瘤切除术。例2，女。57岁。主诉左腰痛不适1个月。CT检查示左。肾上腺、左肾、胰体肿物，直径分别为2．7、4．5、2．1cm。例3，女，39岁。查体发现左肾上腺占位1个月。CT检查示左肾上腺3．0cm×4．0cm实性占位，增强后肿块明显强化。既往有小脑、脊髓血管母细胞瘤及双侧肾癌手术史。例4，女，41岁。B超发现双肾肿瘤1个月入院。CT检查示左肾、左肾上腺、右。肾、胰腺肿物，直径分别为4．0、3．0、1．5、2．0cm。1个月前行7刀治疗多发脑部肿瘤。结果4例均手术治疗。例1行左肾根治性切除术，病理报告肾血管周细胞瘤，随访6个月右肾未见异常。例2行左肾、左肾上腺、胰体尾及脾切除，病理报告肾透明细胞癌、胰岛细胞瘤、肾上腺囊肿，随访3个月MRI检查发现脊髓肿瘤。例3行左。肾上腺肿瘤摘除术，病理报告嗜铬细胞瘤，随访2年未见肿瘤复发。例4行左肾、左肾上腺切除，病理报告肾透明细胞癌、肾上腺嗜铬细胞瘤，3周后剜除右肾肿瘤，病理报告透明细胞癌，随访1年肿瘤未复发。结论VHL可并发多器官肿瘤，较大中枢神经系统肿瘤可手术切除，多发较小的实性肿瘤可放疗。直径〈3cm肾癌可密切随访、择期处理；直径〉3cm肾癌首选保留肾单位手术。肾上腺肿瘤首选腺瘤摘除术，避免肾上腺全切术。腹部多器官肿瘤一期手术安全可行。

Objective To discuss the surgical management of yon Hippel-Lindau（VHL） syndrome. Methods The clinical data of four VHL patients who were clinically diagnosed from March 1999 to October 2006 were reviewed. The first patient was a 56-year-old man with the chief complaint of hypodynamia and low serum glucose as 2.37 mmol/L. CT scan showed three masses in the the left kidney. He had a history of cerebral haemangioblastoma ten years before. The second case was a 57- year-old woman with the chief complaint of lumbago in the left side. CT scan showed masses in the left kidney, adrenal gland and pancrease. The third case was a 39-year-old woman with the chief complaint of an accident mass in the left adrenal gland. She had the history of cerebellar hemangioblastoma 9 years before, spinal hemangioblastoma resection 7 years before. The fourth case was a 41-year-old woman, she was found brain tumors and cured by gamma radiation abalation. Bilateral renal masses were found by B-ultrasound one month later, CT scan showed four masses in the both kidneys, left adrenal gland and pancreas. Results All 4 cases underwent surgical approach. The first case underwent radical nephrectomy which pathological report was PEComa of kidney. The blood glucose was normal one week later. The second case underwent resection of the left adrenal gland, kidney, pancreatic body and tail and spleen. Pathological report was clear cell carcinoma, islet cell tumor and adrenal cyst. Three months later she was found spinal hemangioblastoma and refused treatment. The third case underwent adrenalectomy in the left side and pathological report was adrenal pheochromocytoma. There was not tumor recurrence during 2 years＇ follow-up. The nephreetomy and adrenalectomy was performed for the last case whose pathological report was clear cell carcinoma and pheochromocytoma. Three weeks later, tumor enucleating of the right kidney was undertaken; the result was clear cell cancer. During the follow up for one year there was no relapse of tumor. Conclusions For VHL accompanied with multiple organ tumors, surgery resection is the proper approach when tumors of central nervous system is large. Different approaches could be taken to deal with multiple tumors of VHL such as watchful waiting, nephron sparing surgery,