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目的探讨和分析异位嗜铬细胞瘤的临床诊疗特点,提高对该疾病的认识水平。方法回顾性分析1998年9月至2008年9月间病史资料完整并经病理证实的异位嗜铬细胞瘤31例,所有患者均行手术治疗,收集术前及术后随访20~140个月的临床资料。结果 31例异位嗜铬细胞瘤中良性25例,恶性6例;首发症状为高血压者21例,腹痛5例,血尿2例,体检发现者3例。血儿茶酚胺和尿香草扁桃酸(VMA)检测阳性率分别为77.4%(24/31)和61.3%(19/31);B超和CT检查阳性率分别为87.1%(27/31)和96.8%(30/31)。肿瘤多位于腹膜后区域,手术切除肿瘤直径2~11 cm。术后仍有高血压者3例;6例恶性嗜铬细胞瘤患者术后复发4例,其中3例随访期间死亡。结论异位嗜铬细胞瘤临床表现形式多样,定性及定位诊断需结合影像学及实验室检查综合评估。手术切除是最有效的治疗方法。明确的定位诊断,充分的术前准备,严密的麻醉监护对手术实施十分重要。
Objective To investigate and analyze the clinical diagnosis and treatment of ectopic pheochromocytoma and to raise awareness of the disease. Methods Thirty-one cases of ectopic pheochromocytoma with complete history and pathology confirmed from September 1998 to September 2008 were retrospectively analyzed. All patients underwent surgical treatment. The patients were followed up for 20 to 140 months The clinical data. Results 31 cases of ectopic pheochromocytoma were benign in 25 cases and malignant in 6 cases. The first symptom was hypertension in 21 cases, abdominal pain in 5 cases, hematuria in 2 cases and physical examination in 3 cases. The positive rates of blood catecholamines and urinary vanilloids (VMA) were 77.4% (24/31) and 61.3% (19/31), respectively. The positive rates of B-mode ultrasound and CT were 87.1% (27/31) and 96.8% (30/31). More tumors in the retroperitoneal area, surgical resection of the tumor diameter of 2 ~ 11 cm. There were 3 cases of postoperative hypertension; 6 cases of malignant pheochromocytoma recurred in 4 cases, of which 3 died during follow-up. Conclusion The clinical manifestations of ectopic pheochromocytoma are diverse, and the diagnostic and qualitative diagnosis should be combined with the imaging and laboratory tests. Surgical resection is the most effective treatment. Clear positioning diagnosis, adequate preoperative preparation, strict anesthesia monitoring is very important for the operation.