临床病例讨论——复发肾上腺皮质癌  

Recurrent adrenal cortical carcinoma

作　　者：谢玲玎[1] 张金苹[1] 陈晓平[1] 王瑶[1] 邢小燕[1] Xie Lingding;Zhang Jinping;Chen Xiaoping;Wang Yao;Xing Xiaoyan(Department of Endocrinology,China-Japan Friendship Hospital,Beijing 100029,China)

机构地区：[1]中日友好医院内分泌科,北京100029

出　　处：《北京医学》2019年第4期302-305,309,共5页Beijing Medical Journal

摘　　要：目的总结1例复发肾上腺皮质癌(adrenal cortical carcinoma, ACC)的诊断及治疗经验。方法对中日友好医院收治的1例ACC患者的诊疗过程分析总结,并进行相关文献复习。结果本例67岁女性患者,以面部变圆、胡须生长、血压及血糖升高为主要临床表现;实验室检查提示为促肾上腺皮质激素(adrenocorticotropic hormone, ACTH)非依赖性库欣综合征(Cushing syndrome, CS)合并雄激素增多;肾上腺CT示左侧肾上腺巨大肿瘤;手术后确诊ACC;术后1年复发;再次手术后给予米托坦治疗,病情明显好转。结论 ACC临床罕见,病情进展迅速,难以早期发现。对于男性化表现突出,血清睾酮及去氢表雄酮升高的CS患者应考虑ACC可能。关注病理报告中Ki67水平对预后评估有重要作用。尽早启用米托坦治疗可改善患者预后。Objective To summarize the diagnosis and treatment experience of recurrent adrenal cortical carcinoma(ACC).Methods The diagnosis and treatment process of a patient with ACC in China-Japan Friendship Hospital were analyzed and the related literatures were reviewed.Results The main clinical manifestation of the 67 years old female patient was facial rounding,beard growth,hypertension and increased blood glucose level.The test showed ACTH-independent Cushing syndrome(CS)with increased androgen levels.Abdominal CT showed a large mass of left adrenal cortex.After surgery,the pathological diagnosis was adrenal cortical carcinoma.The tumor recurrence occurred one year after surgery.Mitotane was given after reoperation,and the condition improved significantly.Conclusions ACC is a rare clinical disease with rapid progression and difficult to be diagnosed early.The possibility of ACC should be considered in patients with CS with prominent masculine manifestations and elevated serum testosterone and deoxyepiandrosterone.The level of Ki67 in pathological reports is important for prognosis evaluation.Early initiation of mitotane treatment may improve patients'outcomes.

关 键 词：库欣综合征 肾上腺皮质癌 米托坦 

分 类 号：R736.6[医药卫生—肿瘤]