嗜铬细胞瘤诊治50年回顾总结

Diagnosis and treatment of pheochromocytoma:a fifty-year clinical experience

目的探讨嗜铬细胞瘤诊断治疗的变迁。方法回顾性分析50年收治的362例嗜铬细胞瘤临床病例资料。男196例,女166例。年龄7～75岁,平均38岁。病程20d～20年,平均3.9年。根据术前准备用药的变化分为3个时期,对比不同时期的诊断技术、术前扩容方法、麻醉技巧和手术方式,方差分析方法比较各时期的围手术期死亡率。结果第一时期(1955-1975年)60例,诊断依靠尿VMA和腹膜后注气造影,术前仅应用镇静剂,硬膜外麻醉下经开放手术切除肿瘤,围手术期死亡率为8.3%(5/60)。第二时期(1976-1994年)105例,诊断手段以尿儿茶酚胺和超声、CT为主,术前应用酚苄明常规扩容准备,采用全麻,开放手术死亡率降至1.0%(1/105)。第三时期(1995-2004年)197例,均查尿儿茶酚胺、超声和CT,131I间位碘代苄胍(131IMIBG)核素扫描作为诊断该病的特异性手段,术前准备采用酚苄明和乌拉地尔,指端微循环图像判断,常规术式为全麻下腹腔镜肿瘤切除术,围手术期死亡率为0.5%(1/197)。结论嗜铬细胞瘤诊断方法日益丰富和全面。术前扩容准备方法规范化和多样化。微创手术成为切除嗜铬细胞瘤的主要方法,围手术期死亡率明显下降。

Objective To review and discuss the changes of diagnostic techniques and therapeutic modalities of pheochromocytoma during the past fifty years. Methods The clinical data of 362 patients （196 men and 166 women; age range,7 -75 years;mean age,38 years） with pheochromocytoma from January 1955 to July 2004 were retrospectively analyzed. The disease course ranged from 20 d to 20 years with a mean of 3.9 years. According to the premedication,the past 50-year period was divided into 3 stages;and the diagnostic techniques,premedication, anesthetic skills,surgical procedures and perioperative death rate were compared among the 3 stages. Results There were 60 patients in the first stage （1955 - 1975） and their diagnoses were made on urine VMA and retroperitoneal air-contrast imaging. They had no premedication except for tranquilizers. The therapeutic modality was open surgery of resecting tumors under peridural anesthesia. The perioperative mortality was 8.3% （5/60）. The second stage （ 1976 - 1994） included 105 patients and they were diagnosed mainly by 24-h urine catecholamine, ultrasonography and CT. Phenoxybenzamine was taken as the regular premedication and open surgery general anesthesia was used; the mortality decreased significantly to 1.0% （ 1/105 ）. The third stage （ 1995 - 2004） consisted of 197 cases. In addition to 24-h urine catecholamine, ultrasonography and CT, 131^I-MIBG scan was used as the specific diagnostic technique for pheochromocytoma widely. The premedication was phenoxybenzamine and urapidil. The microcirculation imaging analysis was used to judge the volume expansion preparation. Laparoscopic resection of the tumor under general anesthesia was adopted as the regular means. The perioperative mortality was 0.5% （ 1/ 197）. Conclusions The diagnostic techniques for pheochromocytoma have become enriched and mature. The premedication has been standardized and included more medicines. The microinvasive surgery has become the current method for resecting tumors,and the perioperative mortality has become significantly decreased.