异位ACTH综合征的研究进展

Ectopic ACTH syndrome

能引起异位促肾上腺皮质激素（ACTH）综合征的肿瘤种类较多,β连环蛋白相关的Wnt信号通路、Nouch信号通路、P21活化的蛋白激酶3在异位分泌ACTH肿瘤的发生、发展中起重要作用,促阿片黑素细胞皮质素原基凶启动子区去甲基化与肿瘤异位分泌激素密切相关.在诊疗方面,血清嗜铬粒蛋白A的测定大大提高了异位ACTH综合征的阳性预测率.除手术治疗外,联合用药较单独使用生长抑素或选择性多巴胺受体激动剂更为有效.

The heterogeneity of the ectopic ACTH syndrome （EAS） has already been described. Wnt signaling pathway, Notch signaling pathway and PAK3 may be involved in the development of neuroendocrine tumor while hypomethylation in the promoter region of POMC gene correlates with ectopic overexpression in thymic carcinoids. The measurement of chromogranin A increases the positive predictive value of EAS. Besides surgery, given dopamine receptor agonist and somatostalin in combination could be more effective in controlling cortisol excess than given in alone.