多发性内分泌瘤病2A型13例临床分析

摘要

目的 探讨多发性内分泌瘤病2A型(MEN-2A型)的临床特点和治疗方法.方法 回顾性分析1988年至2011年我院收治的13例MEN-2A型患者的临床资料.13例均为嗜铬细胞瘤伴甲状腺髓样癌,无甲状旁腺功能亢进.其中8例甲状腺髓样癌发病早于嗜铬细胞瘤,5例同时伴发嗜铬细胞瘤.均行嗜铬细胞瘤切除,其中9例行双侧肾上腺肿物切除,4例行腹腔镜肾上腺肿物切除术；10例行甲状腺癌根治术,颈部淋巴结清扫,3例行甲状腺单侧叶切除术.结果 术后病理检查,肾上腺均为嗜铬细胞瘤,其中恶性倾向3例；甲状腺均为甲状腺髓样癌.13例术后均随访至2012年6月,存活10例,死亡3例,死因均为甲状腺髓样癌远处转移.结论 此病罕见,手术是治疗该病的惟一方法,当嗜铬细胞瘤与甲状腺髓样癌同时存在时,首先应切除嗜铬细胞瘤.%Objective To review clinical characteristics and treatment of multiple endocrine neoplasia type 2A (MEN-2A).Methods The clinical data of 13 patients with MEN-2A admitted to our hospital between 1988 and 2011 were retrospectively reviewed.All 13 cases were diagnosed as pheochromocytoma with medullary thyroid carcinoma,presenting no hyperparathyroidism,including 8 cases who had medullary thyroid carcinoma before pheochromocytoma and 5 cases who had medullary thyroid carcinoma and pheochromocytoma simultaneously.All 13 cases underwent resection for pheochromocytoma; 9 cases had bilateral adrenal resection,including 4 cases undergoing laparoscopic resection for pheochromocytoma.Thyroidectomy with bilateral dissection of regional lymph nodes was performed in 10 patients,and nodule enucleation was performed in 3 remaining patients.Results Adrenal pathology reported pheochromocytoma in all cases,including 3 malignant cases.Thyroid pathology reported medullary thyroid carcinoma in all cases.All 13 patients were followed-up visit,10 cases survived and 3 died from distant metastasis of medullary thyroid carcinoma.Conclusions MEN-2A is a rare disease.Surgery is the only treatment for this disease ; when patients have both pheochromocytoma and medullary thyroid carcinoma,to first remove the pheochromocytoma is preferable.