儿童库欣病1例报告及文献复习

Cushing's disease in children( report of 1 case and review of literature)

目的探讨儿童库欣病(CD)的临床特点及诊断、治疗方法。方法对南京大学医学院附属鼓楼医院神经外科2016年3月收治的1例儿童库欣病的临床表现、血液生化、影像学、病理学资料及显微外科手术方法和预后进行回顾性分析。结果患儿的肿瘤位于垂体下缘,与垂体分界尚清楚。一侧岩下窦与外周静脉血促肾上腺皮质激素(ACTH)的基础值之比≥2。MRI检查示,病变T_1WI表现为等信号或稍低信号,T_2WI表现为略高信号,增强后垂体下缘可见多发小结节样低信号影。术中见多个肿瘤样组织,暗红色,质韧。术后病理检查诊断为垂体腺瘤。免疫组化结果示,肿瘤细胞表达ACTH(散在+),PRL(-),FSH(部分+),TSH(-),P53(-),Ki167(小于1%+)。结论儿童垂体促肾上腺皮质激素微腺瘤有一定的特征性临床表现。增强MRI检查具有诊断参考作用,岩下窦采血检测ACTH有确诊意义。经蝶显微手术切除垂体腺瘤是治疗的最佳手段。

Objective To explore the diagnosis and treatment of Cushing＇s disease（ CD） in children.Methods The clinical and imaging manifestations,the pathologic features and microsurgical outcome of 1 child patient with Cushing＇s disease（ CD） admitted by our hospital in March,2016 were analyzed retrospectively,and the related literature was reviewed and compared. Results The tumor was located in the lower edge of the pituitary,the base value of the lower sinus and peripheral vein ACTH is greater than or equal to 2,with clear boundaries to the pituitary tissue. Equal or slightly lower signals on MRI T1-weighted images（ T_1WI） and T_2-weighted images（ T_2WI） showed slightly higher signal. The lower edge of the pituitary showed multiple nodules lower signals was noted on contrastenhanced T_1WI and T_2WI. Multiple tumor like tissues,dark red,and Tenacity detected during the operation.Postoperative pathology showed pituitary adenoma,immunohistochemistry indicated that the tumor cells expression of ACTH（ ＋,scattered）,PRL（-）,FSH（ ＋,part）,TSH（-）,P53（-）,Ki167（ less than 1% ＋）. Conclusion Cushing＇s disease in children has certain characteristics in clinical,with contrast-enhanced MRI has a certain secondary reference effect,blood sampling from inferior petrosal sinus is of definite diagnostic significance.