Heikkinen E S, Akerblom H K
J Pediatr Surg. 1977 Apr;12(2):157-63. doi: 10.1016/s0022-3468(77)80002-x.
Two children, aged 14 yr, with multiple pheochromocytomas are presented. Both patients had a positive family history. In the preoperative aortographies the intra-adrenal pheochromocytomas of both patients were well visualized, but not the extra-adrenal tumors of the first case. Chlorpromazine as an adrenergic blocking agent was successfully used in the preoperative treatment. Postoperative catecholamine excretion in the first case was repeatedly slightly increased indicating residual pheochromocytoma. In addition to the bilateral adrenal pheochromocytomas, multiple islet cell adrenomas and cholecystolithiasis were revealed at the operation of the second case. After bilateral adrenalectomy and total pancreaticoduodenectomy, regular follow-up examinations were carried out for 28 months. Hyperparathyroidism and signs of possible medullary thryoid carcinoma were discovered. Thus the patient had an unique pattern of MEA syndrome.
本文报告了两名14岁患有多发性嗜铬细胞瘤的儿童。两名患者均有阳性家族史。术前主动脉造影清晰显示了两名患者肾上腺内的嗜铬细胞瘤，但第一例患者的肾上腺外肿瘤未显示。氯丙嗪作为一种肾上腺素能阻滞剂成功用于术前治疗。第一例患者术后儿茶酚胺排泄量反复轻度增加，提示存在残留嗜铬细胞瘤。第二例患者手术时除发现双侧肾上腺嗜铬细胞瘤外，还发现多发胰岛细胞瘤和胆结石。双侧肾上腺切除及全胰十二指肠切除术后，进行了28个月的定期随访检查，发现了甲状旁腺功能亢进及可能的甲状腺髓样癌迹象。因此，该患者具有MEA综合征的独特模式。
