多发性内分泌腺瘤2A的诊治(附2例报告)

Multiple endocrine neoplasia type 2A (Report of 2 cases)

目的:提高多发性内分泌腺瘤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;1例行嗜铬细胞瘤切除及甲状腺全切并淋巴结清扫术,1例行双侧嗜铬细胞瘤切除及甲状腺穿刺活检术。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。

Objective：To explore the methods of diagnosis and treatment for multiple endocrine neoplasia type 2A. Methods： Two cases of MEN2A were reported . The clinical features, diagnosis and treatment of MEN2A was presented and discussed with reviewing the literature. Results： The diagnosis of medullary thyroid carcinoma and pheochromocytom was established on P, ultrasonography, CT. MRI and 131I-MIBG scanning. The plasma calcitonin, plasma and urine catecholamine levels were noted abnormal. The patients were cured by surgery. Conclusions：MEN2A is a rarely seen disease. The diagnosis depends on the necessary checks of endocrine and imaging studies.Radical tumor resection is the treatment of choice. Screening in families with genetic tests and earlier surgical therapy are very important for the treatment.