腹部异位嗜铬细胞瘤33例诊治分析

Diagnosis and treatment of extra-adrenal pheochromocytoma： a report of 33 cases

目的 总结腹部异位嗜铬细胞瘤的临床特点及诊治经验.方法 回顾性分析中国医科大学附属第一医院2005年1月至2012年12月收治的33例腹部异位嗜铬细胞瘤的临床资料.对其临床表现、诊治及预后进行分析.结果 异位嗜铬细胞瘤占同期收治嗜铬细胞瘤的16.2％（33/204）.其中男13例,女20例,年龄（45±15）岁.以腹部不适等临床表现为主诉入院的14例（42.4％）;有头晕、视物模糊等症状的8例（24.2％）;1例膀胱嗜铬细胞肿瘤患者表现为排尿时心慌.10例（30.3％）没有明显的临床表现.33例患者均行CT检查,8例患者凭影像学表现做出诊断.1例无法切除仅作病理诊断,其余均行肿瘤切除术,1例联合左肾切除,1例联合胰尾脾切除.本组无围手术期死亡病例,预后良好.结论 异位嗜铬细胞瘤缺乏典型临床表现和特异性实验室检查手段,应重视其影像学表现,切除手术后预后良好.

Objective To study the diagnosis and management of extra-adrenal pheochromocytoma.Methods A retrospective clinical study was done on 33 patients of extra-adrenal pheochromocytoma treated at our department.Results Extra-adrenal pheochromocytoma accounted for 16.2％ （33/204） of all pheochromocytoma patients admitted between Jan 2005 and Dec 2012.There were 13 males and 20 females.The mean age at diagnosis was 45 years,with a range from 17 to 81 years.14 patients were hospitalized for abdominal discomfort,8 patients suffered from typical hypertension symptoms including headache and dizziness and blurred vision,one of bladder pheochromocytoma was found paroxysmal hypertension and 10 patients had no obvious clinical symptoms.All the patients underwent CT scan,and 8 extra-adrenal pheochromocytoma were discovered.11 patients were given circulation expansion therapy.Most of the tumors were located at bladder（3 case）,renal hilus （7 case） and para-aorta（23 case）.There were two patients with multiple tumors.In 26 cases,blood pressure fluctuated when touching tumor during operation.All the patients were treated with surgical operation.Except for 1 biopsy,32 patients underwent total resection of tumor including a case combined resection with left kidney and a case with the spleen.There were no perioperative mortality.Postoperative follow up showed very good prognosis,with no death.Conclusions Compared with imaging examination,clinical manifestations and laboratory tests were less evaluable for the diagnosis of extra-adrenal pheochromocytoma.Proper preoperative preparation and precise operation is essential for the prognosis.