儿童嗜铬细胞瘤(附18例报告)

Pheochromocytoma in children (report of 18 cases)

目的 提高儿童嗜铬细胞瘤的诊治水平。 方法 报告 18例儿童嗜铬细胞瘤临床资料。男 12例 ,女 6例。年龄 6～ 14岁 ,平均 10岁。主要临床表现为剧烈头痛 ,心悸 ,多汗和视物模糊等 ;血压持续增高者 10例 ,持续增高伴发作性加剧者 7例 ,发作性增高者 1例。平均血压 185 /130mmHg(1mmHg =0 .133kPa) ,最高达 2 6 0 /190mmHg。 2 4h尿VMA增高者占 89% (16 /18) ,血、尿儿茶酚胺增高者占 92 % (12 /13) ,腹膜后充气造影 3例均阳性 ,B超阳性者占 94 % (15 /16 ) ,CT 14例均阳性 ,MRI 2例均阳性。 结果 本组 18例占同期收治嗜铬细胞瘤的患者 11.6 % (18/15 5 )。在妥善围手术期处理下 ,18例均行手术治疗 ,手术过程顺利。切除肿瘤 4 0～ 15 0 g。术后随访 1～ 2 0年 ,血压恢复正常者占 94 % (17/18) ;1例 2年后肿瘤复发 ,诊断为恶性嗜铬细胞瘤 ,再次手术后 1年死亡。 结论 儿童嗜铬细胞瘤临床表现不典型 ,对儿童高血压患者应进行相应内分泌和影像学检查。手术切除为最佳治疗方法 ,妥善的围手术期处理很重要。术后血压恢复正常率高于成人。

Objective To evaluate the diagnosis and treatment of pheochromocytoma in children. Methods Eighteen children with pheochromocytoma were diagnosed and treated from 1980 to 2000.The primary clinical manifestations included severe headache,blurring of vision,palpitation,excessive perspiration.Sustained hypertension occurred in 10 cases,sustained hypertension accompanied by episodic exacerbation in 7 and episodic hypertension in 1.The mean blood pressure was 185/130 mmHg(1 mmHg=0.133 kPa),with the highest up to 260/190 mmHg.VMA was increased in 89% (16/18) of the cases,and blood and urinary catecholamine was increased in 92% (12/13).Retroperitoneal pneumography was performed in 3 cases,CT in 14 cases and MRI in 2 cases,with all positive findings.B-ultrasonography showed positive in 94%(15/16). Results This series of 18 cases accounted for 11.6% of the pheochromocytoma cases admitted homochronously (18/155).All the 18 cases underwent surgery with the help of proper peir-operative management.The pheochromocytomas (comfired by pathology) were successfully extirpated with the tumor weight ranging from 40 to 150 g.All the cases were followed up for 1 to 20 years.Their blood pressure returned to normal in 94%(17/18).Only 1 case had relapse 2 years later with diagnosis of malignancy,and died in the following year. Conclusions Since the clinical manifestations of pheochromocytoma in children are atypical,endocrinological and imaging examinations are necessary to children with hypertension.Surgical excision is the best option,and oppropriate perioperative management is extremely important.The outcome of surgical management in children is better than that in the adults.