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Von Hippd-lindau氏病是罕见的常染色体显性遗传病,可在许多器官发现囊肿和肿瘤,多发或双侧肾细胞癌即常伴发生Lund过去8年的42例VHL中10例有肾细胞癌。患者年龄平均33岁,13次手术共发现62个肿瘤,全部属Ⅰ期,57个直径<3cm。30个由术前放射检查诊断,18个CT检出,30个血管造影检出。作MRT4例仅发现2个肿瘤。行一例肾切除2次,部分肾切除11次,治疗共切除62个肿瘤,37个系肿瘤从受累较少肾脏剖出。8例行双侧探查,无双侧肾切除。血肌酐手术前、后无明显变化,无其他重大并发症,手术平均失血1300ml,平均手术时间4.5小时。
Von Hippd-lindau’s disease is a rare autosomal dominant disease with cysts and tumors found in many organs. Multiple or bilateral renal cell carcinoma is often associated with Lund. Of the 42 cases of VHL in the past 8 years, 10 have renal cells. cancer. The patients were 33 years old. A total of 62 tumors were found in 13 surgeries. All of them belonged to phase I, 57 diameters <3 cm. Thirty patients were diagnosed by preoperative radiological examination, 18 CT scans, and 30 angiograms. Only 2 tumors were found in 4 MRT cases. A nephrectomy was performed twice, partial nephrectomy was performed 11 times, and a total of 62 tumors were resected and 37 tumors were dissected from less involved kidneys. Eight patients underwent bilateral exploration without bilateral nephrectomy. There was no significant change in blood creatinine before or after surgery, and there were no other major complications. The average blood loss was 1300ml and the average operation time was 4.5 hours.