本文選題：ACTH綜合征　切入點(diǎn)：異位　出處：《北京大學(xué)學(xué)報(bào)(醫(yī)學(xué)版)》2017年04期

【摘要】：目的:探討異位促腎上腺皮質(zhì)激素(adrenocorticotrophic hormone,ACTH)綜合征的診斷與外科治療。方法:對1996年1月至2016年12月北京協(xié)和醫(yī)院收治的異位ACTH綜合征57例的臨床資料進(jìn)行總結(jié),其中男32例、女25例,年齡11~68歲,平均32歲。血ACTH顯著升高,平均77.6 pmol/L(16.5~365.6 pmol/L,正常值為10.1 pmol/L),垂體MRI未見占位,CT檢查雙側(cè)腎上腺均有不同程度彌漫性或結(jié)節(jié)樣增生。按照不同治療方案選擇分為3組:A組25例,未發(fā)現(xiàn)異位腫瘤的異位ACTH綜合征,行雙側(cè)或單側(cè)腎上腺切除的手術(shù)治療;B組16例,發(fā)現(xiàn)異位腫瘤行腫瘤根治性切除術(shù);C組16例,非手術(shù)治療。分析不同治療方法療效及預(yù)后。結(jié)果:57例患者中有40例隨訪6個(gè)月至10年,A組因病情兇險(xiǎn)程度不同、術(shù)中情況各異,采取的治療方式也有差別,部分為分期雙側(cè)腎上腺切除或僅行單側(cè)腎上腺切除術(shù),其中4例死于嚴(yán)重庫欣綜合征(Cushing’s syndrome)導(dǎo)致的糖尿病、肺部感染,18例存活至今,3例失訪,存活率為81%(18/22)。B組16例行異位腫瘤根治性切除者,5例術(shù)后0.5~6.0年死于腫瘤復(fù)發(fā),3例存活至今,8例失訪,存活率為37.5%(3/8)。C組4例放射療法加化學(xué)藥物治療者1年內(nèi)均死于腫瘤轉(zhuǎn)移、糖尿病或肺部感染,6例單純化學(xué)藥物治療者均于1年內(nèi)死于肺部感染,另6例失訪,無1例存活。結(jié)論:異位ACTH綜合征的治療難度大,靶腺切除是有效的治療方法,尤其適用于庫欣綜合征臨床癥狀嚴(yán)重而原發(fā)腫瘤無法定位者。
[Abstract]:Objective: to investigate the diagnosis and surgical treatment of ectopic adrenocorticotrophic hormone (ACTH) syndrome.Methods: the clinical data of 57 cases of ectopic ACTH syndrome treated in Peking Union Hospital from January 1996 to December 2016 were reviewed. There were 32 males and 25 females, aged 1168 years with an average age of 32 years.Serum ACTH was significantly increased (mean 77.6 pmol/L(16.5~365.6 / L, normal value was 10.1 pmol / L). No diffuse or nodular hyperplasia of bilateral adrenal glands was found in pituitary MRI.According to different treatment schemes, 25 patients in group A were divided into 3 groups. There were no ectopic ACTH syndrome, 16 cases were treated with bilateral or unilateral adrenalectomy, 16 cases were found to be treated with radical resection of ectopic tumor and 16 cases were treated with radical resection of tumor.Non-surgical treatment.The curative effect and prognosis of different treatment methods were analyzed.Results 40 of 57 patients were followed up for 6 months to 10 years. The patients in group A had different degrees of illness, different intraoperative conditions and different treatment methods, some of which were bilateral adrenalectomy or only unilateral adrenalectomy.Among them, 4 cases died of diabetes caused by severe Cushing's syndrome (Cushingssyndrome), 18 cases of pulmonary infection have survived and 3 cases have not been visited.The survival rate was 81% 18 / 22%. B group (16 cases) with radical excision of ectopic tumor, 5 cases died of tumor recurrence from 0.5 to 6.0 years after operation, 3 cases survived and 8 cases lost their visit. The survival rate was 37.5% / 8%. 4 cases in group C died of tumor metastasis within 1 year after radiotherapy and chemotherapeutic therapy.All 6 patients with diabetes or pulmonary infection died of pulmonary infection within one year, 6 patients lost their visit and none survived.Conclusion: the treatment of ectopic ACTH syndrome is difficult, target adenectomy is an effective treatment method, especially for Cushing syndrome patients with severe clinical symptoms and the primary tumor can not be located.
【作者單位】： 中國醫(yī)學(xué)科學(xué)院北京協(xié)和醫(yī)院北京協(xié)和醫(yī)院泌尿外科;
【分類號】：R699.3

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