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。     

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。     

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。     

促肾上腺皮质激素非依赖性肾上腺皮质大结节样增生的治疗

目的 提高促肾上腺皮质激素( adrenocorticotropic hormone,ACTH)非依赖性肾上腺皮质大结节样增生( ACTH-independent macronodular adrenal hyperplasia,AIMAH)患者的治疗效果. 方法 回顾性分析2000至2011年收治并确诊的17例AIMAH患者的临床资料,包括亚临床AIMAH3例、临床AIMAH 10例以及高危AIMAH患者4例.影像学均表现为双侧肾上腺明显增大,伴多个大小不等结节,呈典型“生姜样”改变.3例亚临床AIMAH患者ACTH水平降低,血、尿皮质醇正常或轻度升高,l mg过夜地塞米松抑制试验被抑制,无典型库欣综合征(Cushing syndrome,CS),仪存在高血压、糖尿病等非特异性症状.临床AIMAH以及高危AIMAH患者表现为CS症状,血、尿皮质醇升高,血浆ACTH降低,皮质醇昼夜分泌节律消失,大、小剂最地塞米松抑制试验均不被抑制.高危AIMAH患者表现为骨质疏松2例,肝功能不全2例,心肺功能不全3例,严重高血压4例.亚临床AIMAH患者行对症治疗,临床AIMAH患者行手术治疗,高危AlMAH患者行酮康唑(800 mg/d)加手术治疗. 结果 3例亚临床AIMAH患者经对症治疗,血压、血糖平稳出院,随访3个月～3年,内分泌指标检查正常.7例临床AIMAH患者行单侧肾上腺肿块加同侧肾上腺部分或者全部切除,6～9个月后CS症状完全消失.2例临床AIMAH患者同时行双侧肾上腺全切除术,其中1例因肾上腺皮质功能危象而死亡,另1例出现肾上腺皮质功能减退症状,给予糖皮质激素替代治疗,随访5年,生化指标显示正常,无Nelson综合征.l例临床AIMAH患者先后行单侧肾上腺全切术,随访10年,常规补充糖皮质激素,无Nelson综合征.4例高危AIMAH患者经酮康唑治疗后行右侧肾上腺全切术,术后继续服用酮康唑(400 mg/d或800 mg/d),1～2个月后血、尿皮质醇恢复正常,随访1～3年,生化指标正常. 结论 不同亚型AIMAH 应采取不同治疗方法.对于亚临床AIMAH,重点在于对症治疗,随访期间宜定期进行肾上腺影像学以及内分泌功能检查,一日进展为临床AIMAH,首选单侧肾上腺全切术.高危AIMAH应先通过药物抑制皮质醇合成,患者能够耐受于术后尽快切除一侧肾上腺.对于临床AIMAH,单侧肾上腺切除是一种有效的治疗方法.     

肾上腺大结节增生的外科治疗

目的探讨肾上腺大结节增生(AIMAH)的临床特点与诊治方法。方法总结17例　AIMAH　患者的临床表现、内分泌检查、影像与病理特点以及治疗和预后。男9例,女8例,年龄15～66岁,平均42岁,双侧15例,左右侧各1例。有典型库欣综合征临床表现者10例,另7例表现为高血压或糖尿病症状;实验室检查显示　ACTH<2.2 pmol/L,血皮质醇325.1～1876.8 nmol/L,24 h　尿游离皮质醇71.7～2332.2 nmol/24 h,皮质醇分泌节律消失,大、小剂量地塞米松抑制试验不被抑制12例。CT或MRI显示双侧或单侧不规则结节或团块样增大,直径最大5 cm。结果16例行手术治疗,病理报告为肾上腺皮质结节样或腺瘤样增生。其中行单侧肾上腺切除术10例,双侧肾上腺全切除术4例,双侧肾上腺次全切除术2例。双侧肾上腺全切及次全切除6例症状完全缓解,未复发,未发生尼尔森综合征;单侧肾上腺切除者,库欣症状缓解4例,1例未缓解;5例亚临床AIM- AH,术后血压均降至正常,但糖尿病症状未缓解。结论AIMAH作为皮质醇症一种独立的临床亚型,具有自主性皮质醇分泌、肾上腺多发大结节、双侧肾上腺切除术后不发生尼尔森综合征等临床特征。单侧肾上腺切除可缓解多数AIMAH症状,对于症状不缓解或复发者可行双侧肾上腺次全切或全切。     

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。     

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.     

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。     

肾上腺皮质增生类疾病的诊断及外科治疗(附180例报告)

目的 探讨肾上腺皮质增生类疾病的临床诊断及外科治疗方法. 方法 肾上腺皮质增生患者180例.男74例,女106例.年龄6～76岁,平均40岁.其中皮质醇增多症152例,醛固酮增多症28例.180例均行肾上腺手术治疗,术后病理检查均符合肾上腺皮质增生诊断.回顾分析患者临床表现特点、实验室检查结果、肾上腺CT影像特点及疗效. 结果 180例中库欣病(CD)107例,促肾上腺皮质激素(ACTH)非依赖性肾上腺大结节增生(AIMAH)22例,异位ACTH所致肾上腺皮质增生(EAAH)19例,原发性色素结节样肾上腺皮质增生(PPNAH)4例,特发性醛固酮增多症(IHA)28例.实验室检查前4组平均24 h尿游离皮质醇(24 h UFC)分别为287.6(95.2～535.7)、307.9(24.8～808.2)、852.5(102.5～3127.0)、564.3(243.8～1124.6)μg,皮质醇分泌节律消失比例分别为99%(102/103)、92%(11/12)、100%(17/17)、100%(4/4);AIMAH、EAAH、PPNAH组大、小剂量地塞米松抑制试验(DDST)均不被抑制,CD组大剂量DDST可被抑制.28例IHA患者中血钾降低(<3.5 mmol/L)17例,尿钾升高(>30 mmol/24 h)15例,肾素及醛固酮测定符合诊断.180例患者肾上腺CT检查均提示病变侧肾上腺体积增大.102例皮质醇增多症患者行单侧肾上腺切除,术后1周内CD、AIMAH、EAAH、PPNAH组平均24 h UFC分别为157.4(56.2～233.5)、117.9(22.5～418.5)、756.7(116.5～1137.0)、164.3(124.6～422.6)μg.21例IHA患者行单侧肾上腺病灶切除,术后血压恢复正常8例(38%),较术前下降13例(62%).57例行双侧肾上腺手术治疗(双侧全切39例,一侧全切一侧次全切16例,双侧次全切除2例),其中皮质醇增多症患者术后1周复查24 h UFC为12.8～98.5μg,平均77.6μg;IHA患者血压正常.106例(59%)随访4～158个月,平均32个月,库欣病症状及高血压症状均有改善. 结论 需要手术治疗的肾上腺皮质增生类疾病首选单侧肾上腺全切治疗,后期根据临床观察可选择对侧肾上腺次全切除或全切除,可获得较好治疗效果.     

腹腔镜单侧肾上腺切除术治疗8例原发性双侧肾上腺大结节样增生患者的疗效随访

目的探讨行腹腔镜单侧肾上腺切除术的8例原发性双侧肾上腺大结节样增生(PBMAH)患者的治疗效果。方法回顾性分析香港大学深圳医院2015年1月至2020年12月收治的8例确诊为PBMAH患者的临床资料。其中男性5例,女性3例,平均年龄50(34～61)岁。定性诊断有4例为促肾上腺皮质激素(ACTH)非依赖性库欣综合征(CS),另外4例为ACTH非依赖性亚临床库欣综合征(SCS)。完善内分泌、生物化学、病理及影像学检查。所有患者术前均分别行多学科讨论,手术先切除增生明显或者增生体积较大的一侧肾上腺,围手术期和术后均予以激素替代治疗。术后定期随访患者血常规、肝功能、肾功能、血钾、肾上腺相关激素水平与功能试验、垂体及肾上腺MRI/CT等。结果 8例均行后腹腔途径的腹腔镜单侧肾上腺切除术治疗,右侧5例,左侧3例,手术顺利,均无并发症发生。术前肾上腺增强CT提示7例患者双侧肾上腺弥漫性增大且伴有多发结节,1例患者6年前切除左侧肾上腺故表现为右侧肾上腺弥漫性增大伴结节。病理结果提示肾上腺皮质结节状增生5例,肾上腺皮质腺瘤3例。平均随访30.5个月,2例典型CS临床表现和体征的患者,症状得到缓解。8例患者术后的血压和体重指数(BMI)较术前有明显改善,24h的尿皮质醇水平及血钾均正常,双侧肾上腺均被切除的1例患者术后行终身激素替代治疗。结论腹腔镜单侧肾上腺切除术治疗PBMAH安全有效,能改善患者的CS临床症状,推荐为临床一线治疗方案。若行双侧肾上腺全切术,术后需要终身激素替代治疗。     

Results of unilateral adrenalectomy in subclinical Cushing's syndrome due to adrenocorticotropic hormone‐independent macronodular adrenal hyperplasia

Adrenocorticotropic hormone (ACTH)‐independent bilateral adrenocortical macronodular adrenocortical hyperplasia (AIMAH) is a rare cause of Cushing's syndrome (CS). Traditionally, bilateral adrenalectomy with subsequent lifetime steroid replacement has been considered to be the treatment of choice. In the present study, we evaluated the long‐term results of unilateral adrenalectomy in subclinical CS (SCS) due to AIMAH, with regard to the main laboratory and clinical abnormalities. Two patients with confirmed SCS due to AIMAH underwent unilateral laparoscopic adrenalectomy to reduce the cortisol‐secreting tissue. These procedures were successfully conducted in both cases without open conversion, and no surgery‐related morbidity occurred. In both cases, the size of the remaining adrenal gland appeared quite stable, and neither of the patients showed a Cushingoid appearance. Unilateral adrenalectomy achieved satisfactory and prolonged control of cortisol secretion, and also reduced the risk of metabolic disorders and cardiovascular disease after surgery. It can be a safe and effective treatment for SCS due to AIMAH, while maintaining the patient's quality of life.     

The Role of Unilateral Adrenalectomy in Corticotropin-Independent Bilateral Adrenocortical Hyperplasias

Background

The objective of the present study was twofold: to demonstrate our experience with unilateral adrenalectomy in the treatment of adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS) caused by bilateral adrenocortical hyperplasias, and to evaluate the long-term results as evidenced by the main laboratory and clinical findings.

Methods

From February 2000 to August 2009, unilateral adrenalectomy was performed on 27 patients with ACTH-independent CS and bilateral adrenocortical hyperplasias, including 14 patients with ACTH-independent macronodular adrenal hyperplasia (AIMAH) and 13 patients with primary pigmented nodular adrenocortical disease (PPNAD). Signs and symptoms of CS, endocrine examinations, and radiographic imaging were evaluated preoperatively and postoperatively.

Results

At a median follow-up of 69 months (range: 23–120 months) for AIMAH and 47 months (range: 16–113 months) for PPNAD, 25 patients were cured by unilateral adrenalectomy. Serum cortisol level, daily urinary free cortisol (UFC), and plasma ACTH regained the normal range in both AIMAH and PPNAD patients at monthly follow-up visits; the circadian serum cortisol rhythm returned to normal, and a normal responsiveness to overnight low-dose dexamethasone administration (LDDST) became obvious. Both systolic and diastolic blood pressure (BP) levels were significantly reduced: 85 % of patients recovered normal BP levels, and the remaining patients need antihypertensive drugs, but at a reduced dose. No surgery-related morbidity occurred, and there was no sign of further enlargement of the residual adrenal gland after successful unilateral adrenalectomy. One patient with PPNAD and another patient with AIMAH with similar weights and sizes of the bilateral adrenals needed contralateral adrenalectomy.

Conclusions

Unilateral adrenalectomy may be the suitable treatment for selected patients with AIMAH and PPNAD. It can achieve long-term remission of CS and improve glycemic control and BP values.     

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.     

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     

The Role of Unilateral Adrenalectomy in ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)

Background ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing’s syndrome. Bilateral adrenalectomy is considered the treatment of choice, with subsequent lifetime steroid replacement, but unilateral adrenalectomy has been recently proposed to reduce the secreting tissue. This prospective study was designed to evaluate the long-term results of unilateral adrenalectomy in AIMAH concerning the main laboratory and clinical abnormalities and the patient’s quality of life. Methods Seven consecutive patients with confirmed AIMAH underwent unilateral adrenalectomy of the largest gland. ACTH and cortisol levels, arterial blood pressure (BP), glycometabolic parameters, and patient’s subjective perception of health-related quality of life (by the SF-36 questionnaire) were measured preoperatively and postoperatively. Results No surgery-related morbidity occurred. One patient with a large contralateral adrenal enlargement required a completion adrenalectomy after 7 months because of persistent hypercortisolism. At a median follow-up of 53 (range, 27–68) months, the remaining six patients were cured, because serum and urinary free cortisol levels significantly decreased and ACTH increased, thus regaining the normal range. Both systolic and diastolic BP levels significantly reduced: 50% of patients definitively became normotensive, and the remaining patients reduced the need for antihypertensive treatment; 40% of patients suffering from preoperative diabetes were cured, whereas 40% reduced the need for hypoglycemizing drug. SF-36 evaluation of the health-related quality of life confirmed a significant amelioration. Conclusions Unilateral adrenalectomy of the largest gland can be an effective and safe treatment for AIMAH in case of asymmetric involvement. It may achieve long-term remission of Cushing’s syndrome and improve BP values, glycemic control, and patient’s quality of life.     

Simultaneous Bilateral Laparoscopic Adrenalectomy for Adrenocorticotropic Hormone-Independent Macronodular Adrenal Hyerplasia: Report of a Case

Cushing's syndrome caused by adrenocorticotropic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) is an extremely rare disease, which shows bilateral macronodular adrenal hypertrophy and autonomous cortisol production. We herein report a case of AIMAH treated successfully by minimally invasive simultaneous bilateral laparoscopic adrenalectomy. A 73-year-old woman with hypertension, diabetes mellitus, and osteoporosis was referred to our hospital because of an incidentally found huge bilateral adrenal mass. An abdominal computed tomography scan showed large bilateral adrenal glands with multiple nodules. A diagnosis of AIMAH was made and a simultaneous bilateral laparoscopic adrenalectomy was thus performed. The total operation time was 310 min and blood loss was 70 g. Both glands were hypertrophic (right 5 × 3 cm, 48.5 g and left 4 × 2 cm, 39.2 g) and consisted of multiple golden yellow macronodules. The postoperative course was uneventful. A simultaneous bilateral adrenalectomy for AIMAH performed by an experienced surgical team is therefore considered to be a safe and minimally invasive procedure.     

ACTH-independent bilateral macronodular adrenocortical hyperplasia (AIMAH): a case report]

We report a case of adrenocorticotropic hormone (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH). A 54-year-old Japanese man was admitted to our hospital for further examination of obesity and hypertension. Endocrinological studies showed that plasma cortisol was high (22.5 micrograms/dl) without diurnal rhythm, and plasma ACTH was low. Two or 8 mg of dexamethasone did not suppress the plasma cortisol levels. Abdominal computed tomography revealed nodular hyperplasia of bilateral adrenal glands. Adrenal scintigraphy showed the positive uptake of 131I-adosterol to bilateral adrenal glands. Brain magnetic resonance imaging revealed no abnormalities. He was diagnosed as having Cushing's syndrome with bilateral adrenal hyperplasia, and bilateral adrenalectomy was performed. Left and right adrenal glands were 52 g and 35 g, respectively, and were occupied by yellow nodular lesions. Histologically, hyperplastic lesions were composed of clear cells. Finally he was diagnosed with AIMAH.     

The Clinical Conundrum of Corticotropin-independent Autonomous Cortisol Secretion in Patients with Bilateral Adrenal Masses

Background Management of patients with bilateral adrenal masses and corticotropin (ACTH)-independent Cushing syndrome (CS) or subclinical CS is problematic. We report our experience with adrenal venous sampling (AVS) in the evaluation of 10 patients with bilateral masses who had ACTH-independent CS or subclinical CS. Patients and Methods Ten patients (9 women, 1 man, mean age 56.4 years) with bilateral adrenal masses and ACTH-independent CS (n = 3) or subclinical CS (n = 7) underwent AVS. Autonomous cortisol secretion was documented in all cases with suppressed serum ACTH concentrations and lack of cortisol suppression with dexamethasone administration. Adrenal venous sampling was performed on the second day of dexamethasone administration. Cortisol and epinephrine levels were measured from each adrenal vein (AV) and from a peripheral vein (PV). Results Mean (± SD) maximal diameter of the adrenal masses on computed tomography was 3.3 ± 1.3 cm (range: 1.2–6.0 cm). Successful catheterization was confirmed with AV:PV epinephrine gradients. A cortisol AV:PV gradient >6.5 was consistent with a cortisol-secreting adenoma in 11 adrenal glands; 5 patients had clinically important bilateral autonomous cortisol hypersecretion, 3 had bilateral cortisol-secreting adenomas, and 2 had ACTH-independent macronodular adrenal hyperplasia. Adrenal venous sampling-guided adrenalectomy was completed in all 10 patients—2 patients had total bilateral adrenalectomy and 2 others had subtotal bilateral adrenalectomy. During a mean follow-up of 36.1 months (range: 0.7–123 months), CS or clinically important cortisol secretory autonomy did not recur. Conclusions Adrenal venous sampling contributed to the localization of autonomous hypercortisolism in the setting of ACTH-independent CS or subclinical CS in patients with bilateral adrenal masses. Presented at the Annual Meeting of the International Association of Endocrine Surgeons, Montreal, Canada, August 26–29, 2007. J. A. Carney is an Emeritus Member of the Department of Laboratory Medicine and Pathology.