儿童嗜铬细胞瘤的诊断与治疗(附6例分析及文献复习)

Diagnosis and treatment of pheochromocytoma in children: analysis and literature review of 6 cases

的：提高儿童嗜铬细胞瘤的诊断和治疗水平。方法：回顾性分析13年来6例患儿的临床资料及复习文献。结果：24小时尿香草苦杏仁酸（Vanillylmandelicacid, VMA）增高4例；血儿茶酚胺（Catecholamine, CA）增高6例；超声、CT、MRI检查均有阳性发现。肾上腺嗜铬细胞瘤4例，肾上腺外嗜铬细胞瘤2例。全部患儿均在全麻下手术成功切除肿瘤，术后病理检查均证实为嗜铬细胞瘤。术后随访1年～13年，5例无瘤存活至今，1例复诊发现肿瘤多发转移，家长放弃进一步治疗，2月后死亡。结论：虽然术前充分准备如扩容、控制血压，术中减少对肿瘤的挤压避免术中突然出现恶性高血压、控制术后低血压是至关重要的，但手术切除肿瘤是根治方法；组织病理学鉴别肿瘤良恶性很困难；若儿童伴恶性高血压合并心率失常、心悸、视力障碍或腹痛，需警惕此病。

Objectlve：To promote the diagnosis and treatment in children with pheochromocytoma. Methods：Clinical data of six patients with pheochromocytoma during thirteen years were analyzed retrospectively and literatures were reviewed. Results ： Four cases had an increase of vanilla mandelic acid（VMA） within 24 h;six cases had an increase of blood catecholamine（CA） and were found positive mass by ultrasound,CT and MRI examination;four cases had adrenal mass;two cases had extra-adrenal mass（bladder）. Surgical excision was performed in all of cases under general anesthesia and pheochromocytoma was confirmed postoperatively by histologic appearance. Patients were followed up for 1-13 years;five cases had tumor-free survival;only one case revealed tumor metastases by referral examination and died two months later due to disagreement of further treatment. Conclusion ： The radical treatment of pheo- chromocytoma is surgical excision. It is crucial to perform preoperative preparations including volume dilatation and management of hypertension,to avoid intraoperative extrusion of tumor and intraoperative occurrence of severe hypertension,and to control blood pressure postoperatively. Differentiating benign from malignant pheochromocytoma by histologic appearance is difficult. In case of child with malignant hypertension, arrhythmia, palpitation, visual disturbance or abdominal pain, pheochromocytoma need to be considered.