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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 investigate the diagnosis and treatment of multiple endocrine neoplasia type Ⅱb (MENⅡb). Methods: The diagnosis, treatment and prognosis of one case of delayed diagnosis and treatment of MENⅡb were reviewed and reviewed. Results: The 38-year-old female had left adrenal pheochromocytoma as the first symptom 5 years ago and underwent resection of pheochromocytoma. Three years ago, the right lobe tumor of the thyroid was found, which was misdiagnosed as thyroid adenoma and followed up for 6 months Before, once again due to the right side of the adrenal gland cell tumor and admitted to the hospital, patients with all-body type and multiple neuroma, familial history prompted a certain degree of family hereditary, CT showed typical medullary thyroid carcinoma (MCT) calcification , Diagnosed MEN Ⅱ b type, followed by pheochromocytoma resection and MCT radical surgery, postoperative recovery. Conclusion: MENⅡb is a rare disease. To strengthen the understanding of this disease is the key to improve the diagnosis. The treatment of advanced pheochromocytoma should be followed by MCT. The monitoring and treatment of postoperative MCT recurrence and metastasis is to improve long-term The key to survival.