协和医学杂志

2015, v.6(06) 432-436

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定位诊断困难的支气管类癌致库欣综合征一例及文献复习
Difficult Locating Diagnosis in a Case of Cushing's Syndrome Due to Bronchial Carcinoid: Case Report and Literature Review

袁涛;冀晋;王曦;卢琳;伍学焱;程欣;陈野野;陆召麟;
YUAN Tao;JI Jin;WANG Xi;LU Lin;WU Xue-yan;CHENG Xin;CHEN Ye-ye;LU Zhao-lin;Department of Endocrinology,Key Laboratory of Endocrinology of Ministry of Health,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Nuclear Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & P

摘要(Abstract):

目的探讨库欣综合征病因为支气管类癌所致异位促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)综合征的定位诊断方法。方法报道1例定位诊断困难的49岁女性库欣综合征患者,收集其临床资料,对其定位诊断过程和方法进行回顾性分析。结果患者曾经被误诊为库欣病并接受经蝶窦垂体手术。库欣综合征的症状在垂体手术后仍持续存在。经过6个月的随访和数周的酮康唑治疗,胸部CT显示右肺内一个直径约5 mm小结节在9个月内无明显变化。生长抑素受体显像和氟脱氧葡萄糖-正电子发射断层显像(fluorodeoxyglucose-positron emission tomography,FDGPET)不能确定肺内结节为分泌ACTH的肿瘤。岩下窦静脉取血结果表明病因为异位ACTH综合征。胸腔镜下右肺中叶切除术后,血ACTH、皮质醇和24 h尿游离皮质醇均降至正常。组织学证实该结节为典型支气管类癌,免疫组织化学染色示ACTH阳性。结论岩下窦静脉取血对于异位分泌ACTH肿瘤的定位诊断非常重要,对于疑诊异位ACTH综合征的患者应进行岩下窦静脉取血联合不同的影像学诊断技术来定位病灶,必要时可行探查手术。
Objective To explore the localization diagnosis method of Cushing's syndrome caused by ectopic adrenocorticotropic hormone( ACTH) syndrome due to bronchial carcinoid. Methods Based on the clinical data,we retrospectively analyzed the process and method of localization diagnosis in a 49-year-old female patient with Cushing's syndrome which was difficult to localize the etiology. Results The patient had been misdiagnosed as Cushing's disease,for which she had accepted transsphenoidal pituitary surgery. The symptoms of Cushing's syndrome persisted after the pituitary surgery. After six months' follow-up and treatment with ketoconazole for several weeks,chest CT revealed that the size of one small nodule( about 5 mm in diameter) in the right lung didn't change for 9 months. The somatostatin receptor scintigraphy and fluorodeoxyglucose-positron emission tomography( FDG-PET) could not confirm that the pulmonary nodule was the tumor producing ACTH. The results of inferior petrosal sinus sampling confirmed that the etiology was ectopic ACTH syndrome.After removing the right middle lobe of the lung through thoracoscopic surgery,plasma ACTH,serum cortisol,and 24-hour urinary free cortisol were reduced to normal. The nodule was histologically diagnosed as a typical bronchial carcinoid containing immune-reactive ACTH as shown in immunohistochemical staining. Conclusions Inferior petrosal sinus sampling may be important for localizing the lesion of ectopic ACTH production,which,combined with different imaging techniques,should be considered in patients suspected for ectopic ACTH syndrome. Exploratory surgery may be sometimes needed.

关键词(KeyWords): 支气管类癌;库欣综合征;异位促肾上腺皮质激素综合征;岩下窦静脉取血
bronchial carcinoid;Cushing's syndrome;ectopic adrenocorticotropic hormone syndrome;inferior petrosal sinus sampling

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作者(Author): 袁涛;冀晋;王曦;卢琳;伍学焱;程欣;陈野野;陆召麟;
YUAN Tao;JI Jin;WANG Xi;LU Lin;WU Xue-yan;CHENG Xin;CHEN Ye-ye;LU Zhao-lin;Department of Endocrinology,Key Laboratory of Endocrinology of Ministry of Health,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Cardiology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & Peking Union Medical College;Department of Nuclear Medicine,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences & P

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