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目的探讨von-Hippel-Lindau(VHL)综合征并发肾癌的临床诊断和治疗水平。方法回顾分析2008年9月2008年10月分别收治的2例VHL综合征并发双肾多发肿瘤患者的临床诊治过程并结合文献进行讨论。结果 2例患者均为女性。例1为36岁,因腰痛不适3个月入院。既往8年有左眼视网膜血管母细胞瘤手术史。入院后双肾CT检查发现双肾肿瘤伴囊肿,考虑诊断为VHL综合征并发双侧肾癌。患者因故拒绝手术治疗,随访至今,患者病情无明显变化。例2为41岁,因B型超声发现右肾占位4年,反复肉眼血尿2年入院。4年前行小脑蚓部血管母细胞瘤切除术。入院后CT检查显示右肾巨大实性肿瘤伴多发囊肿,左肾多发性小的囊实性占位;胰腺多发小囊肿。诊断为VHL综合征并发右侧肾癌,行右肾根治性切除。手术后病理检查为右肾透明细胞癌,手术后随访至今病情稳定。结论 VHL综合征并发肾癌临床少见,若患者表现为双侧、多发性、囊实性肾肿瘤,应考虑VHL综合征并发肾癌。及时正确诊断并根据患者病情制定个体化治疗方案,尽量避免肾切除,保留肾单位,手术结合影像学观察是主要治疗策略。
Objective To investigate the clinical diagnosis and treatment of von-Hippel-Lindau (VHL) syndrome complicated with renal cell carcinoma. Methods The clinical diagnosis and treatment of 2 patients with VHL syndrome complicated with multiple nephrotic syndrome who were admitted to our hospital from September 2008 to October 2008 were retrospectively analyzed and discussed in the literature. Results Both patients were female. Example 1 was 36 years old and was admitted to hospital for 3 months because of back pain. The past eight years have left eye retinoblastoma operation history. Double-kidney CT examination after admission found that renal tumors with cysts, consider the diagnosis of bilateral renal cell carcinoma with VHL syndrome. Patients refused surgery for some reason, follow-up so far, no significant change in the patient’s condition. Example 2 is 41 years old, because of B-mode ultrasound right kidney occupies 4 years, repeated hematuria 2 years hospitalized. 4 years ago, cerebellar vermis hemangioblastomy. CT examination after admission showed a large solid tumor of the right kidney with multiple cysts, multiple small cystic left renal solid mass; multiple small cysts of the pancreas. Diagnosis of VHL syndrome with right renal cell carcinoma, right renal radical resection. Postoperative pathological examination of right renal clear cell carcinoma, postoperative follow-up has been stable. Conclusions The clinical manifestations of renal cell carcinoma complicated with VHL syndrome are rare. If the patients manifest as bilateral, multiple, solid cystic renal tumors, consider renal syndrome with VHL syndrome. Timely correct diagnosis and development of individualized treatment according to the patient’s condition, try to avoid the nephrectomy, nephrons remain, surgery combined with imaging observation is the main treatment strategy.