多发性内分泌腺瘤2A的诊治(附2例报告)

目的　提高对多发性内分泌腺瘤2A(MEN2A)的认识及诊治水平。方法　总结 2 例 MEN2A的诊治经验,并结合文献进行讨论。结果　B超、CT、MRI及131I 间 碘苄胍(MIBG)检查发现甲状腺肿块及嗜铬细胞瘤,血清降钙素及血、尿儿茶酚胺测定异常;例1 行嗜铬细胞瘤切除及甲状腺全切并淋巴结清扫术,随诊至今无复发;例 2 行双侧嗜铬细胞瘤切除及甲状腺穿刺活检术,术后随访9个月,效果良好。结论　行内分泌及影像学检查可诊断此病,肾上腺嗜铬细胞瘤切除及甲状腺全切并淋巴结清扫术是治疗的主要手段,高危家族的基因检测筛选及早期手术是治愈本病的关键。

Diagnosis and treatment of multiple endocrine neoplasia type 2A (report of 2 cases)

Objective To explore the methods of diagnosis and treatment of multiple endocrine neoplasia type 2A. Methods Two cases of MEN2A were reported. The clinical features, diagnosis and treatment of MEN2A were reviewed and presented. Results The diagnosis of medullary thyroid carcinoma and pheochromocytom was established on B-ultrasonography, CT, MRI and 131I-MIBG scanning. Plasma calcitonin, plasma and urine catecholamine levels were found elevated. The patients were cured by operation. Conclusion MEN2A has been rare. The diagnosis depends on necessary endocrine and imaging examinations. Adrenotumorectomy and total thyroidectomy with radical cervical lymphectomy are the major choices. Screening in families with high risk and early open operation are very important in the treatment.