多发性内分泌腺瘤病2A型中嗜铬细胞瘤的诊治

The treatment of pheochromocytoma in multiple endocrine neoplasia type ⅡA

目的通过分析2A型多发性内分泌腺瘤（MEN2A）的临床特点和治疗方法 ,提高对MEN2A的认识水平。方法回顾性分析我院2008~2015年收治的4例2A型多发性内分泌腺瘤患者资料,分析临床治疗方法和效果。结果 4例均行嗜铬细胞瘤切除,3例行甲状腺根治术,术后4例患者的MEN2A型症状均消失。结论手术是治疗该病的唯一方法,当嗜铬细胞瘤与甲状腺髓样癌同时存在时,应先尽快切除嗜铬细胞瘤。腹腔镜嗜铬细胞瘤切除术是治疗MEN2A中嗜铬细胞瘤的理想方法,该病易复发,再次手术难度大,但仍可行腹腔镜下原入路嗜铬细胞瘤切除术。

Objective To improve the acknowledge of multiple endocrine neoplasia type ⅡA （MEN ⅡA） by analyzing the clinical characteristics and treatments of MENIIA. Methods We reviewed the clinical data from 4 cases of MEN ⅡA hospitalized from year 2008 to 2015. The clinical treatments and outcomes were analyzed. Results All 4 cases were underwent laparoscopic pheoehromocytoma resection. 3 cases were underwent radical thyroidectomy. The MEN ⅡA symptoms of all 4 cases were disappeared postoperation. Conclusion Surgical procedure is the only treatment for this disease. When pheochromocytoma and medullary thyroid carcinoma are coincidenee,pheochromocytoma resection is suggested as soon as possible. Laparoscopic pheochromocytoma resection is the ideal method for pheoehromocytoma in the MEN IIA. This disease has a high relapse rate and reoperation is difficult. But it is still feasible to do repeatedly laparoscopic pheochromocytoma resection by the original approach.