多发性内分泌肿瘤Ⅱb型——附1例报告并文献复习  

Multiple endocrine neoplasia type 2b——report of a case undiagnosed for many years and review the literature

作　　者：王立明[1] 赵永福[1] 汤洪波[1] 马灵斐[1] 杨春明[1] 孙卫兵[2] 范治璐[2] 

机构地区：[1]大连医科大学附属第二医院普通外科暨器官移植中心,辽宁大连116027 [2]大连医科大学附属第二医院泌尿外科,辽宁大连116027

出　　处：《中国现代普通外科进展》2004年第1期55-58,共4页Chinese Journal of Current Advances in General Surgery

摘　　要：目的 :探讨提高多发性内分泌肿瘤Ⅱb型 (MENⅡb)诊治水平和方法。方法 :通过 1例延误诊断多年MENⅡb病例诊治经验分析及文献复习 ,提出其诊断、治疗、预后的有关问题。结果 :38岁女性 ,5年前以左侧肾上腺嗜铬细胞瘤为首发症状 ,行嗜铬细胞瘤切除术 ;3年前发现甲状腺右叶肿物 ,误诊为甲状腺腺瘤而随诊观察 ;半年前 ,再次因右侧肾上腺嗜咯细胞瘤而入院 ,患者伴有类马凡体型及多发性神经瘤 ,家族史提示具有一定的家庭遗传性 ,CT显示了典型的甲状腺髓样癌 (MCT)钙化 ,确诊MENⅡb型 ,先后行嗜铬细胞瘤切除和MCT根治术 ,术后康复出院。结论 :MENⅡb型是一种罕见疾病 ,加强对本病的认识是提高诊断的关键 ,治疗上应先行嗜铬细胞瘤切除后行MCT根治术 ,术后MCT复发和转移的监测与治疗是提高长期生存率的关键。Objective:To explore and improve the diagnosis and treatment of multiple endocrine neoplasia,MEN Ⅱb.Methods:By the analysis on diagnosis and treatment of one case of MEN Ⅱb undiagnosed for many years despite its typical phenotype and review the literature.Results:38 yeas old woman,who was admitted to the hospital for left pheochromocytoma five years ago,and underwent left adrenalectomy.The patient presented with a thyroid mass three years ago,and was diagnosed for thyroid adenoma without further treatment.The patient was admitted to the hospital again for right pheochromocytoma half one years ago.Further examinations showed that the patient presented marfan's contour,and calcinosis was found in thyroid mass.Multiple ganglioneuroma were found on the back and lip.The patient was diagnosed with MEN Ⅱb and was cured by right adrenalectomy and total thyroidectomy with routine removal of lymph nodes.Conclusion:MEN Ⅱb is a rare hereditary disorder.The better understanding of clinical features of MEN Ⅱb is quite helpful for diagnosis.The pheochromocytoma should be firstly operated,and then perform thyroidectomy.Follow up on MTC is important after operation.

关 键 词：神经纤维瘤 内分泌腺肿瘤 嗜铬细胞瘤 甲状腺肿瘤 

分 类 号：R736[医药卫生—肿瘤]