【摘要】 目的探讨肺非典型类癌伴异位促肾上腺皮质激素(ACTH)综合征的临床病理特征及诊断标准与鉴别诊断。方法回顾性分析1例肺非典型类癌伴异位ACTH综合征临床病理特征及免疫组织化学表型,并文献复习。结果患者,女性,60岁,颜面浮肿潮红,双下肢无力症就诊。血ACTH 73.04 pg/ml。CT示右肺下叶后基底段结节影。病理特征:"右肺下叶"周围型肿物,最大径2.1 cm,切面灰粉色,质软,周界较清;镜下观察,肿瘤细胞呈巢团状、小梁状或菊形团样排列,细胞中等或偏小,细胞核深染,形态略不规则,核分裂象平均3个/10HPF。免疫组化:CK、ACTH、NSE、Syn、CgA(+),Ki-67约10%(+)。病理诊断:肺非典型类癌伴异位ACTH综合征。结论肺非典型类癌伴异位ACTH综合征少见,需结合临床病史、血清学检查、病理学特征及免疫组化表型综合分析诊断,该病变患者预后良好。更多还原

【Abstract】 Objective To investigate the clinicopathological characteristics,and the diagnostic criteria and differential diagnosis of ectopic adrenocorticotropic hormone(ACTH) syndrome caused by atypical pulmonary carcinoid tumors.Methods A case of ectopic adrenocorticotropic hormone(ACTH) syndrome caused by atypical pulmonary carcinoid tumors was retrospectively analyzed about the clinicopathologic features and immunohistochemical phenotype with the literature review.Results This study described a 60-year-old female patient presenting with facial edema,and flushing and bilateral lower limb weakness,and plasma ACTH 73.04 pg/ml,and CT showed a nodus in the inferior lobe of right lung.Gross pathologic examination showed that peripheral tumor with distinct boundary was located in the inferior lobe of right lung.The largest dimension of tumor was 2.1 cm,and the cut surface was gray-pink with soft texture.Histological structure showed that the nests or trabecular pattern of various sizes.The tumor cells were middle or small with irregular and hyperchromatic nuclei.Immunohistochemistry showed the positive expression of CK,ACTH,NSE,Syn and Cg A of the tumor cell clusters,respectively,and the positive expressed rate of Ki-67 was 10%.Based on the characteristic clinical and morphology and results of immunohistochemistry,a diagnosis of ectopic ACTH syndrome caused by atypical pulmonary carcinoid tumors was made.Conclusion A rare diagnosis of ectopic ACTH syndrome caused by atypical pulmonary carcinoid tumors is appearance based on aforesaid results.Further,we suggest that the patient may heve unfavourable prognosis.更多还原