2型多发内分泌腺瘤病的诊断及外科治疗

目的探讨2型多发内分泌腺瘤病的临床特点、治疗方法及疗效。方法回顾分析我院1980～2002年诊治的8例2型多发内分泌腺瘤病患者的临床特点、诊断及治疗方法与疗效。结果7例有阵发性高血压,5例最高血压超过200mmHg(1mmHg=0．133kPa),3例查体发现肿瘤。经B超、CT及24h尿儿茶酚胺检查确定诊断,2A型6例,2B型2例;表现为甲状腺髓样癌合并嗜铬细胞瘤伴或不伴甲状旁腺腺瘤或增生(6例),1例伴多发黏膜神经瘤,1例为嗜铬细胞瘤伴有马凡综合征;双侧肾上腺肿瘤7例;分期行肾上腺与甲状腺、甲状旁腺结节切除或次全切除术。术后平均随访9年,效果良好。结论2型多发内分泌腺瘤病的诊断主要依赖相应的内分泌检查及B超和CT检查,手术是主要的治疗手段,当嗜铬细胞瘤与其他肿瘤同时存在时,宜首先切除嗜铬细胞瘤。

The clinical characteristics and therapy of multiple endocrine neoplasia type 2

Objective To study the clinical characteristics and treatment of multiple endocrine neoplasia(MEN) type 2 Methods The clinical features, diagnosis and treatment of from 1980 to 2002 8 cases of multiple endocrine neoplasia type 2 from Peking Union Medical College Hospital were reviewed and analyzed in clinical features,diagnosis and treatment retrospectively Results Seven cases were with paroxysmal hepertension,the highest blood pressure was over 200 mm Hg (1 mm Hg=0 133 kPa) Tumor was found in 3 cases The diagnosis was confirmed by B ultrasonography, CT and urine catecholamine test:six cases with MEN 2A 2 with MEN 2B Six cases were medullary carcinoma of thyroid with or without parathyroidoma or hyperplasia, 1 with multiple mucosal neuromata One case was pheochromocytoma with marfan's syndrome;7 cases werw with bilateral adremaltumor Total ectomy or subtotal ectomy was performed on different stages on adrenal gland,parathyroid tubercle Average follow up was 9 years And the feedback was good Conclusions The diagnosis of multiple endocrine neoplasia type 2 depends on endocrine biochemical tests, B ultrasonography and CT When the pheochromocytoma and the other tumor exists at the same time, operation is the primary treatment, and it is better to remore the pheochromocytoma at the first