肾上腺手术在治疗皮质醇增多症中的疗效评价(附90例报告)

目的：提高皮质醇增多症的治疗水平。方法：总结90例皮质醇增多症的临床资料以及随访情况。90例患者均有库欣综合征的临床表现,内分泌检查提示高皮质醇状态。71例患者影像学检查发现肾上腺肿块,其中左侧32例,右侧34例;双侧肾上腺肿块5例。19例患者显示双侧肾上腺结节样增生。结果：90例患者均行肾上腺手术,其中60例行肾上腺肿块切除加同侧肾上腺部分切除;6例行肾上腺肿块切除加同侧肾上腺全切除;4例行同时行双侧肾上腺全切除术,6例行先后双侧肾上腺全切除术;11例行单侧肾上腺全切除术;3例行先后双侧肾上腺次全切除。病理显示6例原发性色素性皮质结节状肾上腺皮质增生（PPNAD）;5例大结节样肾上腺皮质增生（AIMAH）;57例肾上腺皮质腺瘤;5例肾上腺皮质癌;17例肾上腺皮质结节样增生,其中7例考虑异位ACTH综合征所致。结论：肾上腺皮质腺瘤切除术效果最好,库欣症状改善最显著。迁延难愈的库欣病,单侧肾上腺全切除,在短期内能缓解代谢症状。分时分侧行肾上腺全切除术,对治疗AIMAH和PPNAD更安全、稳定;根据异位ACTH综合征患者症状和病程,可同时行双侧肾上腺全切除或单侧肾上腺全切除。     

库欣综合征208例

目的：提高库欣综合征的诊治水平。方法：分析108例促肾上腺皮质激素(ACTH)非依赖性库欣综合征和100例ACTH依赖性库欣综合征患者的临床资料、诊断和手术效果。结果：ACTH非依赖性和依赖性库欣综合征均有库欣综合征的体征和血或尿皮质醇升高；腺瘤型库欣综合征主要显示大剂量地塞米松抑制试验(HDDST)不抑制和CT发现较小的肾上腺占位病变，99例行腺瘤切除术获治愈；肾上腺皮质癌主要表现瘤体直径＞6cm及DHEAS升高，预后差；大结节样肾上腺皮质增生(AIMAH)的CT主要表现双侧肾上腺大结样增生，行双侧肾上腺切除可获治愈；库欣病可被HDDST抑制，CT显示双侧肾上腺增生或无变化，对69例垂体无阳性发现的患者行一侧肾上腺全切除加另一侧肾上腺次全切除症状缓解，20例轻型行一例肾上腺切除加垂体放疗能获长期症状缓解，肾上腺全切分期进行较安全；异位ACTH综合征主要表现ACTH明显升高及发现分泌ACTH肿瘤。结论：对库欣综合征应行鉴别诊断，对不同类型库欣综合征采用适当手术方法可获较满意的手术效果。     

ACTH 非依赖性肾上腺皮质大结节增生症3例报告

目的：提高ACTH非依赖性肾上腺皮质大结节增生症（AIMAH）的评价水平。方法：回顾性分析3例AIMAH患者的临床资料。结果：3例AIMAH患者除具有库兴综合征的临床和生化特点之外，还有如下特点：大剂量地塞米松抑制试验（HDDST）不被抑制，血浆ACTH低水平，CT显示双侧肾上腺大结节样增生改变，病理检查为双侧肾上腺大结节样或腺瘤样增生，单侧肾上腺切除症状可缓解3－4年，双侧肾上腺切除可治愈，术后无Nelson征。结论：AIMAH具有独特的内分泌、CT和病理学特点，是一种独立的库兴综合征病因。     

促肾上腺皮质激素非依赖性肾上腺皮质大结节样增生的外科治疗

目的 探讨促肾上腺皮质激素(adrenocorticotropic hormone,ACTH)非依赖性肾上腺皮质大结节样增生(ACTH independent macronodular adrenal hyperplasia,AIMAH)的诊治经验. 方法 回顾性分析1972年8月至2010年7月诊治14例AIMAH患者资料.男5例,女9例.年龄26～58岁,平均45岁.临床表现为典型库欣综合征( Cushing syndrome,CS)者10例,表现为体质量增加、高血压或高血糖等非特异性症状者4例.生化检查示皮质醇分泌昼夜节律消失,大、小剂量地塞米松抑制试验均不被抑制.CT检查示双侧肾上腺弥漫性增大伴多发结节.14例均行开放手术治疗,其中单侧肾上腺切除5例;一侧肾上腺全切,对侧肾上腺次全切除6例;双侧肾上腺同时切除3例. 结果 14例病理诊断均为肾上腺大结节样或腺瘤样增生.随访12～120个月,平均69个月,CS症状消失.5例单侧肾上腺切除者术后血、尿皮质醇值恢复正常,对侧肾上腺无明显增大.3例双侧肾上腺切除患者中,1例术后7d发生肾上腺皮质功能危象而死亡,2例术后出现皮质功能减退症状,给予糖皮质激素替代治疗.其他患者病情稳定,无Nelson综合征出现. 结论 AIMAH具有独特的临床症状特点,是CS的一种独立罕见类型.首次手术多行单侧肾上腺切除术,可获较长时间的症状缓解.术后应密切随访患者的皮质醇水平、症状和体征,对症状不缓解或复发者可行对侧肾上腺切除或次全切除.     

ACTH非依赖性肾上腺皮质大结节增生症的诊断与手术处理

目的 总结促肾上腺皮质激素（adrenocorticotrop hichormone，ACTH）非依赖性肾上腺皮质大结节增生症（ACTH independent acronodular drenal yperplasia，AIMAH）的诊治经验。方法 回顾性分析5例AIMAH患者的临床资料。结果 5例患者均具有库欣综合征的临床和生化特点，小剂量、大剂量地塞米松抑制试验均不被抑制，血浆ACTH水平低。4例患者行CT检查示双侧肾上腺大结节样增生改变，所有患者均经病理检查证实为双侧肾上腺大结节样或腺瘤样增生。例1、例2行单侧肾上腺切除后，分别于症状缓解3年和5年后复发，行对侧肾上腺全切除后治愈；例3分期行双侧肾上腺切除后治愈；例4、例5行单侧肾上腺切除后，前者5年症状持续缓解，后者仍在随访中。术后无Nelson综合征。结论 IMAH具有独特的内分泌、CT影像和病理学特点，是一种独立的库欣综合征病因，确诊主要依据病理检查。单侧肾上腺切除可获较长时间的症状缓解，应坚持随访患者的皮质醇水平和症状体征，如有复发可行对侧肾上腺切除。     

ACTH非依赖性双侧肾上腺皮质增生所致库欣综合征的外科治疗

目的探讨促肾上腺皮质激素（adrenocorticotropic hormone,ACTH）非依赖性双侧肾上腺皮质增生的临床特点与诊治经验。方法回顾性分析1980年1月至2011年5月诊治21例ACTH非依赖性双侧肾上腺皮质增生患者资料,包括8例ACTH非依赖性肾上腺皮质大结节样增生（AIMAH）和13例原发性色素性肾上腺结节增生（PPNAD）。男6例,女15例。年龄12～58岁,平均34.3岁。临床表现为典型库欣综合征（Cushings syndrome,CS）者18例,表现为体重增加、高血压等非特异性症状者3例。CT检查提示双侧肾上腺结节样增生改变者14例,2例提示左肾上腺占位;5例肾上腺未见明显异常。双侧肾上腺全切3例;双侧肾上腺次全切除术2例,为初次手术行单侧肾上腺切除,术后库欣症状复发,再次行对侧肾上腺次全切除术;单侧肾上腺切除16例。结果手术标本病理结果报告8例为AIMAH,13例为PPNAD。随访16～120个月,CS症状消失。16例单侧肾上腺切除术后血尿皮质醇、血钾、血糖等均恢复正常,对侧肾上腺无明显增大;3例行双侧肾上腺全切者术后醋酸可的松终身替代治疗,无尼尔森综合征发生;2例肾上腺次全切除者系一侧肾上腺全切术后随访中库欣症状复发,行对侧肾上腺次全切术后症状完全缓解。结论 AIMAH和PPNAD均为库欣综合征中罕见的特殊类型,首次手术可行单侧肾上腺切除术缓解症状。术后应密切随访,对症状不缓解或复发者可行对侧肾上腺切除或次全切除。     

ACTH非依赖性大结节样肾上腺增生(附三例报告)

目的 探讨ＡＣＴＨ非依赖性大结节样肾上腺增生（ＡＩＭＡＨ）的临床病理特点。方法 回顾性分析３例ＡＩＭＡＨ临床资料,结合文献进行讨论。结果 ３例ＡＩＭＡＨ均有主加兴综合征的临床表现及生化异常,特点是小剂量、大剂量地塞米松抑制,血浆ＡＣＴＨ水平低,ＣＴ示双侧肾上腺结节样增在。３例均行双侧肾上腺切除,病理诊断双侧肾上腺结节样或腺瘤样增生,随诊４２～１０８个月库兴综合征未复发,未发生Ｎｅｌｓｏｎ综合征。结     

原发性色素沉着性结节性肾上腺皮质病9例诊治分析

目的:探索原发性色素沉着性结节性肾上腺皮质病(PPNAD)的诊治方案。方法:总结我院2010~2016年9例PPNAD患者的临床资料,包括其临床症状、体征,实验室检查、影像学检查、随访资料等,探讨该种疾病的诊断和治疗。结果:9例患者中8例具备典型库欣综合征体貌,1例患者仅表现为高血压。9例患者均具备促肾上腺皮质激素(ACTH)非依赖性特征,表现为ACTH5pg/ml或大剂量地塞米松抑制试验尿游离皮质醇(UFC)未被抑制。影像学可表现为肾上腺基本正常、双侧肾上腺增粗或小结节改变、单侧肾上腺结节或肿物。7例患者行腹腔镜单侧肾上腺切除,术后随访2例症状复发,遂行腹腔镜对侧肾上腺次全切除术。2例仅表现为单侧肾上腺结节或肿物患者,行腹腔镜肾上腺肿物切除术,术后恢复良好。结论:PPNAD为罕见ACTH非依赖性库欣综合征类型,其可依据高皮质醇血症、ACTH非依赖性、影像学检查等做出诊断,明确诊断需术后病理确定。治疗方式可根据患者的病情程度及术后恢复情况以及实验室指标等进行个体化制定。     

原发性色素沉着性结节性肾上腺皮质病的临床诊治（附15例报告）

目的：揭示原发性色素沉着性结节性肾上腺皮质病（PPNAD）的临床特点与诊治.方法：对15例PPNAD患者的临床资料进行分析.15例中典型库欣综合征14例,仅表现高血压者1例.伴Carney综合征者8例.实验室检查显示ACTH〈2.2 pmol/L,血皮质醇分泌节律均消失,其中0点血浆总皮质醇268.27～686.41nmol/L,24 h尿游离皮质醇（UFC）386.4～3 569.51 nmol/24 h,14例小剂量和大剂量地塞米松抑制试验均不被抑制.CT双侧肾上腺增粗或小结节者8例,肾上腺未见明显异常者7例.术前诊断为垂体腺瘤、库欣病3例,诊断为异位ACTH综合征1例.双侧肾上腺全伞切1例.单侧肾上腺切除术11例,肾上腺次全切术3例.结果：单侧肾上腺平均重5.2 g,病理诊断均为PPNAD.随访8个月～17年,肾上腺全切者症状完全缓解,无复发 肾上腺次全切除者症状缓解,无需皮质激素替代 单侧肾上腺切除症状缓解,但仍存在高皮质醇血症.结论：PPNAD罕见,易误诊.库欣综合征、ACTH非依赖性内分泌特点、影像学双侧肾上腺基本＂正常＂或略增粗或小结节等为其临床诊断要点.单侧肾上腺切除不能完全缓解皮质醇症,肾上腺次全切除可能为合理的治疗策略,复发者肾上腺全切.术后密切随诊,监测Carney综合征其他相关病变.     

柯兴氏病的诊断与经蝶窦垂体手术治疗

1932年Harvey Cushing比较系统地描述了12例柯兴氏综合征,随后,人们对柯兴氏综合征的认识逐步加深。柯兴氏综合征是皮质醇分泌过多所致的症侯群。主要临床表现为满月脸、水牛背、向心性肥胖、紫纹、高血压及糖尿病等。其病因有下列几方面:①垂体分泌过多的促肾上腺皮质激素(ACTH);②异位ACTH产生过多;③促肾上腺皮质激释放因子(CRF)产生过多;④自身肾上腺皮质增生(微结节或大结节);⑤肾上腺腺瘤;⑥肾上腺皮质癌。其中,以垂体分泌过多ACTH所致者即柯兴氏病居多,占70％。柯兴氏病的治疗经历了双     

Subclinical Cushing syndrome due to adrenocorticotropic hormone-independent macronodular adrenocortical hyperplasia: changes in plasma cortisol levels during long-term follow-up

A 63-year-old man with bilateral adrenal tumors was treated for hypertension and diabetes mellitus. Endocrinologic examination during follow-up revealed the production of cortisol to be independent of the regulation of the hypothalamo-pituitary-adrenal axis. Therefore, he was diagnosed as having subclinical Cushing syndrome, and these tumors were removed. Adrenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH) was endocrinologically and histopathologically diagnosed. This is the first report demonstrating that endocrinologic changes of AIMAH can be observed during long-term follow-up.     

儿童肾上腺皮质肿瘤(附13例报告)

目的　提高对儿童肾上腺皮质肿瘤的认识。　方法　肾上腺皮质肿瘤患儿 13例。男8例 ,女 5例。年龄 2岁 6个月～ 16岁 ,平均 8岁。左侧 5例 ,右侧 8例。回顾性总结其临床特点及诊断治疗情况。　结果　皮质腺癌 6例 ,腺瘤 7例。 9例伴高血压 ,其中 2例出现高血压危象 ;11例表现为库兴综合征 ;10例合并性征异常。 13例均行手术治疗 ,7例腺瘤均完整摘除 ;6例皮质腺癌中 ,5例行肿瘤摘除但术中肿瘤破溃 ,1例行部分切除。手术并发肾静脉损伤 2例 ,下腔静脉、门静脉及肾静脉多发损伤伴出血性休克 1例 ,膈肌穿破 1例。 6例皮质腺癌患者中 ,4例术后 1年内死亡 ,2例术后 5个月复发并再次手术。　结论　儿童肾上腺皮质癌多伴雄激素分泌过多而出现性征异常 ,应与先天性肾上腺增生或性早熟鉴别。血管损伤为常见手术并发症。肾上腺皮质腺瘤手术疗效良好而腺癌预后不佳     

Clinical and endocrinological features of adrenocorticotropic hormone- independent bilateral macronodular adrenocortical hyperplasia

PURPOSE: We report clinical findings in 5 patients with adrenocorticotropic hormone independent bilateral macronodular adrenocortical hyperplasia. MATERIALS AND METHODS: In 4 males and 1 female 32 to 61 years old (median age 50) we evaluated clinical symptoms, endocrinological and radiological characteristics, treatment modality and postoperative clinical course. RESULTS: All cases presented with some features of Cushing's syndrome. Endocrinological examination revealed autonomous adrenal cortisol production with suppressed adrenocorticotropic hormone and a loss in the diurnal circadian rhythm of plasma cortisol. Abdominal computerized tomography showed bilateral enlargement of the adrenal glands with multiple nodules. 131Iodine labeled adosterol scintigraphy demonstrated remarkable bilateral uptake by the adrenal glands. The pituitary gland appeared normal on magnetic resonance imaging. Open unilateral complete adrenalectomy and contralateral partial adrenalectomy were performed in patient 1, open bilateral complete adrenalectomy was done in patients 2 and 3, and 2 and 1-stage laparoscopic bilateral complete adrenalectomy was performed in patients 4 and 5. Single removed adrenal glands weighed 32 to 108 gm. (median 60). The histological diagnosis was macronodular adrenocortical hyperplasia in all cases. Postoperative followup was 3 to 90 months. Clinical symptoms of Cushing's syndrome disappeared or improved after surgery in all cases. CONCLUSIONS: Although adrenocorticotropic hormone independent bilateral macronodular adrenocortical hyperplasia is a rare form of Cushing's syndrome, physicians are advised to consider it when diagnosing and treating cases of Cushing's syndrome with enlarged bilateral adrenal glands. Bilateral complete adrenalectomy is currently recommended as the treatment of choice.     

Laparoscopic simultaneous bilateral partial and total adrenalectomy: a longer follow‐up

OBJECTIVE

To examine the feasibility and safety of laparoscopic simultaneous bilateral adrenal surgery (LSBAS) and to compare the results between laparoscopic total adrenalectomy (TA) and partial adrenalectomy (PA).

PATIENTS AND METHODS

Between 1999 and January 2008, 12 patients underwent LBAS in our institution, with TA used in four with Cushing’s disease refractory to pituitary surgery or radiation, in one with occult ectopic adrenocorticotropic hormone (ACTH) Cushing’s syndrome, and one with bilateral adrenal macronodular hyperplasia. Six patients (five with primary aldosteronism and one with Cushing’s syndrome) had bilateral functioning tumours had bilateral PAs.

RESULTS

All 12 operations were completed with no intraoperative complication, conversion, re‐operation or death. The mean (range) operative duration was 323 (180–560) min, and the mean estimated blood loss was 79 (20–200) mL. There was adrenal insufficiency after surgery in three patients. Patients who had a PA had significantly longer surgery than those treated with TA, with a mean (sd ) of 390 (36) vs 255 (27) min.

CONCLUSION

LSBAS is technically feasible; although surgery was longer than TA, bilateral PA in patients with bilateral functioning tumours mitigated the need for life‐long steroid replacement.     

Selective use of steroid replacement after adrenalectomy: lessons from 331 consecutive cases

HYPOTHESIS: Only selected patients require steroid replacement therapy following adrenalectomy. DESIGN: Retrospective review. SETTINGS: University tertiary care center and veterans' hospital. PATIENTS: A total of 331 patients who underwent adrenalectomy by 1 surgeon (Q.-Y.D.) between April 1, 1993, and August 31, 2005. INTERVENTIONS: Laparoscopic, open, and hand-assisted adrenalectomy. Steroid replacement therapy was administered using a standardized hydrocortisone taper protocol. MAIN OUTCOME MEASURES: Indications for adrenalectomy, operative approach, requirement for postoperative steroid replacement, and episodes of acute adrenocortical insufficiency. RESULTS: Of the 331 adrenalectomies, 304 were laparoscopic, 23 were open, and 4 were hand assisted. There were 299 unilateral adrenalectomies and 32 bilateral adrenalectomies performed. Fifty-seven (17%) of the 331 patients required steroid replacement after adrenalectomy. Of the 57 patients requiring steroid replacement, 52 had Cushing syndrome and 5 had bilateral pheochromocytomas. The 52 patients with Cushing syndrome included 16 with pituitary tumors who had failed pituitary resection and/or medical therapy, 14 with unilateral adrenal adenomas, 9 with ectopic corticotropin-secreting tumors who had failed resection and/or medical therapy, 7 with incidentalomas and subclinical Cushing syndrome, 4 with macronodular hyperplasia, and 2 with adrenocortical carcinoma. No patients undergoing unilateral adrenalectomy for non-Cushing adrenal disease required steroid replacement. Four (7%) of the 57 patients receiving steroid replacement had episodes of acute adrenocortical insufficiency following operation and required increased steroid supplementation. There were no cases of acute adrenocortical insufficiency in the 274 patients who did not receive steroid replacement. CONCLUSIONS: Steroid replacement therapy after adrenalectomy should be reserved for patients with Cushing syndrome (overt or subclinical) and patients undergoing bilateral adrenalectomy. Patients undergoing adrenalectomy for unilateral non-Cushing adrenal tumors do not require postoperative steroid replacement.     

肾上腺皮质增生组织中促肾上腺皮质激素的表达

目的 :检测促肾上腺皮质激素 (ACTH)在增生的肾上腺皮质组织中的表达 ,探讨肾上腺皮质球状带增生的发生机制。方法 :采用免疫组织化学SABC染色法检测 18例库欣综合征的肾上腺皮质组织、33例原醛型增生肾上腺皮质组织和 19例正常肾上腺组织ACTH的表达。结果 :原醛型增生肾上腺皮质组织内有丰富的ACTH表达 ,库欣综合征肾上腺皮质组织及正常肾上腺组织内ACTH低表达 ,三者之间ACTH的阳性表达差别有统计学意义 (P <0 .0 1)。结论 :肾上腺内局部表达ACTH可能是肾上腺皮质球状带增生形成的重要因素。