异位ACTH综合征32例诊断和疗效观察

目的 对异位促肾上腺皮质激素(ACTH)综合征病例进行临床分析,为临床诊治工作提供指导。方法 对1990年1月至2003年4月间收治的32例异位ACTH综合征患者资料进行总结。结果 所有病例临床表现和生化测定均符合库欣综合征的诊断。10例患者找到异位肿瘤,诊断明确;4例患者经体表肿物活检或岩下静脉及股静脉插管分段取血查ACTH提示为异位ACTH瘤,诊断基本明确;18例患者经临床及影像学检查高度怀疑异位ACTH综合征。15例(47％)患者因异位肿瘤定位不明确而行双侧或单侧肾上腺全切术,1年存活率60％;7例(22％)患者发现可疑异位肿瘤,未行手术治疗,1年存活率0;10例(31％)患者行根治性肿瘤切除,6例为支气管类癌,4例为胸腺类癌,1年存活率60％。结论 异位ACTH综合征异位肿瘤定位极为困难,仅少数病例可以找到异位肿瘤行手术切除。多数病例无法确定异位肿瘤,双侧肾上腺全切加激素替代治疗是一种有效方法。     

ACTH非依赖性库兴综合征(附89例报告)

目的　总结促肾上腺皮质激素 (ACTH)非依赖性库兴综合征的诊断和治疗方法。　方法　对 89例ACTH非依赖性库兴综合征患者的临床资料进行总结。男 11例 ,女 78例。年龄 13～5 6岁 ,平均 34岁。其中腺瘤型皮质醇症 85例、肾上腺皮质癌 2例、大结节样肾上腺皮质增生 2例。回顾性总结其临床、生化特征 ,影像学特点和治疗方法。　结果　 85例腺瘤型皮质醇症术后效果良好 ,库兴综合征症状消失 ;2例肾上腺皮质癌未能切除者术后分别存活 7和 9个月 ;大结节样肾上腺皮质增生 2例行双侧肾上腺切除后治愈。　结论　血浆ACTH水平、地塞米松抑制试验及B超、CT等影像学检查有利于ACTH非依赖性库兴综合征的诊断和鉴别诊断 ,手术治疗腺瘤型皮质醇症和大结节肾上腺增生可获得较理想的疗效 ,肾上腺皮质癌预后差。     

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。     

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。     

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.     

腹腔镜一期手术治疗双侧肾上腺肿瘤的安全性和疗效分析

目的:探讨腹腔镜一期手术治疗双侧肾上腺肿瘤的安全性和疗效。方法:对17例34侧腹腔镜一期手术治疗双侧肾上腺肿瘤患者的一般情况、手术方法及效果进行回顾性分析。结果:行腹腔镜双侧肾上腺全切术3例,一侧肾上腺全切和另一侧肾上腺部分切除术7例,双侧肾上腺部分切除术7例。平均手术时间136(105~185)min,平均术中出血125(88~260)ml,平均术后住院7(5~8)d。术中无大血管或脏器损伤等严重并发症发生,术后切口感染1例。3例双侧肾上腺全切患者术后均行终身激素替代治疗。术后平均随访16(3~36)个月。高血压等症状消失或较术前好转,未见肿瘤复发。结论:腹腔镜一期手术治疗双侧肾上腺肿瘤安全、可行。     

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.     

异位促肾上腺皮质激素综合征的临床分析

目的 提高异位促肾上腺皮质激素综合征（异位ACTH综合征）诊断和治疗效果。方法 回顾分析1985年7月－1999年7月收治的12例异位ACTH综合征患者的临床表现、诊断和治疗的临床资料。结果 12例患者经内分泌检查诊断为异位ACTH综合症，10例经影像学检查发现原发肿瘤。随访7个月－8年，5例手术切除肿瘤者，3例死亡；3例肾上腺切除者，1例存活至今；4例化疗者均死亡。结论 对有库欣综合征症状的患者，先经内分泌检查确诊为异位ACTH综合征，结合原发肿瘤的症状和体征，对好发肿瘤的部位进行影像学筛查，有助于发现原发肿瘤。对原发肿瘤进行根治性切除，预后较好。     

Surgical strategy in the management of non-small cell ectopic adrenocorticotropic hormone syndrome.

BACKGROUND. Non-small cell ectopic adrenocorticotropic hormone (ACTH) syndrome is a rare cause of hypercortisolism that may require surgery for either curative resection or palliative adrenalectomy. METHODS. We report our surgical experience with 41 patients with ectopic ACTH syndrome and no evidence of small cell lung cancer at initial evaluation. RESULTS. All 41 patients had documented hypercortisolism secondary to ectopic production of ACTH. Based on imaging study results, we determined that 21 patients had localized/resectable disease; eight patients had metastatic disease, and 12 patients had occult disease at examination. Of the 21 patients with localized disease, 16 (76%) were cured of ectopic ACTH by surgery (15 bronchial carcinoid, one pheochromocytoma). Patients with bronchial carcinoid had the greatest probability for cure of ectopic ACTH syndrome, and patients with thoracic primary tumor were more likely to be cured than patients with abdominal primaries. Of the eight patients who had metastatic disease, none were cured of the disease; five patients underwent bilateral adrenalectomy, and three patients were given medical therapy. Only one patient was alive after 5 years. Of the 12 patients who had occult disease, four patients were eventually cured of the disease (three bronchial carcinoid, one thymic carcinoid); one patient died of disease (small cell lung cancer), and seven patients still have occult disease. Nine of 12 patients with occult disease underwent bilateral adrenalectomy for surgical management of hypercortisolism. CONCLUSIONS. This study suggests that the most common primary focus of ectopic ACTH production is within the thorax with 25 of 34 (74%) identifiable tumors originating within either the thymus or bronchus. Adrenalectomy offers excellent palliation of hypercortisolism secondary to either occult or metastatic disease. Patients who initially have localized disease usually have bronchial carcinoids and have a high probability of cure with surgical resection (81%).     

Laparoscopic bilateral adrenalectomy for occult ectopic ACTH syndrome

INTRODUCTION: Ectopic adrenocorticotropic hormone (ACTH) production is responsible for approximately 15% of the cases of Cushing's syndrome. Bilateral adrenalectomy is the most effective treatment for ectopic ACTH syndrome due to occult or disseminated tumors, but the open approach carries substantial morbidity. In this paper, we review our experience with laparoscopic bilateral adrenalectomy for occult ectopic ACTH syndrome. MATERIALS AND METHODS: Adrenalectomies performed by the authors were identified and the outcomes of laparoscopic bilateral adrenalectomies for ectopic ACTH syndrome were examined. Bilateral adrenalectomies were performed sequentially in full lateral decubitus, with patient repositioning between the sides. RESULTS: From 2001 to 2006, the authors performed 16 adrenalectomies in 14 patients, with 11 performed laparoscopically. Two women with occult ectopic ACTH syndrome, refractory to medical management, underwent laparoscopic bilateral adrenalectomies. Operative times were 240 and 245 minutes, including repositioning. One patient underwent a simultaneous wedge liver biopsy for a right lobar lesion. There were no complications. Each patient resumed a regular diet on the first postoperative day. Inpatient hospital stays were 3 days each, mainly for steroid-replacement management. Final pathologic diagnoses were diffuse adrenocortical hyperplasia. Both patients noted a quick improvement in Cushing's syndrome symptoms and signs and were maintained on hydrocortisone and fludrocortisone replacement without incident for over 2 years. CONCLUSIONS: Laparoscopic bilateral adrenalectomy for ectopic ACTH syndrome refractory to medical management can be performed with low morbidity. Symptoms and signs of hypercortisolism rapidly improve postoperatively.     

Gastrinoma associated with common bile duct obstruction and the ectopic production of ACTH.

A case of adrenocortical hyperfunction due to ectopic production of ACTH by a gastrin-producing tumor of the pancreas is described. Cushing's syndrome preceded the appearance of the overt Zollinger-Ellison syndrome by 2 years and was treated by bilateral adrenalectomy. The Zollinger-Ellison syndrome was initially treated with cimetidine, which successfully reduced the secretion of gastric acid. Because the pancreatic gastrinoma continued to grow, causing obstruction of the common bile duct, biliary diversion and total gastrectomy were performed. There is evidence that the pancreatic gastrinoma was the source of the ectopic production of ACTH and possibly secretion. The role of Histamine-2 blocking agents as therapy in the Zollinger-Ellison syndrome is discussed.     

Cushing syndrome due to ectopic adrenocorticotropic hormone secretion

Cushing syndrome (CS) caused by ectopic adrenocorticotropic hormone (ACTH) production (EA) poses major challenges diagnostically by mimicking the pituitary-dependent form of CS and therapeutically by producing severe, life-threatening hypercortisolemia. This retrospective follow-up study describes the clinical characteristics and course of EA in a large referral center. Computer-based cross-index codes for EA, CS, and bilateral adrenalectomy were used to identify patients treated at the Mayo Clinic between 1956 and 1998. EA was confirmed in 106 patients. Gender distribution showed a slight female predominance (61:45). Bronchial carcinoid was the most frequent cause of EA (25%), followed by islet cell cancer (16%), small-cell lung carcinoma (11%), medullary thyroid cancer (8%), disseminated neuroendocrine tumor of unknown primary source (7%), thymic carcinoid (5%), pheochromocytoma (3%), disseminated gastrointestinal carcinoid (1%), and other tumors (8%). No tumor was found in 16% of patients. Altogether, 28 patients were managed medically, and the others underwent curative tumor resection (13 patients) or bilateral adrenalectomy (65 patients). Surgically treated patients had longer survival, but this was most likely affected by treatment bias. The diagnoses of CS and ACTH-secreting neoplasm were usually concurrent, although, there were remarkable cases in which the two conditions were diagnosed several years apart. Curative resection of the tumor producing EA was possible in a small proportion of patients (12%). When curative resection is not possible, patients who are reasonable surgical candidates are likely to benefit from adrenalectomy. Additional experience with bilateral laparoscopic adrenalectomy should increase the number of patients who benefit from adrenal-directed surgery.     

异位ACTH综合征的诊断与外科治疗

目的 探讨异位ＡＣＴＨ综合征的诊断与外科治疗效果。方法 对１９８７年１０月～１９９９年５月收治的异位ＡＣＴＨ综合征异位ＡＣＴＨ综合征１２例的临床资料进行总结，８例行手术治疗，其中２例欠住院发现异位肿瘤者行肿瘤根治性切除术；６例库兴综合征症状严重但未找到异位肿瘤者，行双侧腺上切作术，术后加皮质扩素替代治疗，非手术治疗４例，其中放疗加化疗２例，单纯化疗２例。结果 １２例中１１例随记６个月～１０年，２例     

Bilateral Adrenalectomy for Ectopic Cushing’s Syndrome—Discussions on Technique and Indication

Background Tumors producing adrenocorticotropic hormone (ACTH) or corticotropin releasing hormone (CRH) often remain undiagnosed until severe Cushing’s syndrome appears, and it may be difficult to distinguish from Cushing’s syndrome due to pituitary tumors. Many patients suffer from disease spread, with metastases in the liver or other locations, and the main symptoms may be mineral disturbances, diabetes mellitus, or psychological symptoms from the severe hypercortisolism. Bilateral adrenalectomy may alleviate this situation, but is sometimes a troublesome procedure in these severely ill patients. Methods We have retrospectively investigated 8 patients with ectopic Cushing’s syndrome who have undergone bilateral adrenalectomy at the University Hospital in Uppsala. In addition, another 5 patients who underwent bilateral adrenalectomy for other reasons (recurrent pituitary Mb Cushing or bilateral hyperplasia) were scrutinized for technical considerations. Indications, timing of surgery, and operative procedures were studied to identify signs that may support our approach to management in the future. Results Curative surgery was not possible in any of the cases with ectopic Cushing’s syndrome. Of the 13 operated patients, handport-assisted laparoscopic adrenalectomy was successfully performed bilaterally in 5 patients and unilaterally in combination with contralateral open surgery in 1 patient; conventional open surgery was performed on 7 patients, 3 of which were conversions from intitial handport-assisted procedures. Non-fatal complications occurred in 4 out of 10 patients. Conclusions We conclude that bilateral handport-assisted laparoscopic adrenalectomy is safe, and that all surgical techniques in these severely ill patients may be troublesome and technically demanding. Early surgical intervention may reduce the technical disadvantages. Moreover, bilateral adrenalectomy can substantially reduce the symptoms of Cushing’s syndrome, although effects on mortality are not obvious.     

Endoscopic treatment of bilateral pheochromocytoma in MEN 2A syndrome: case report and review of the literature

The benefits of laparoscopic adrenalectomy for single adrenal lesion have been well documented in literature; less experience though has been reported with simultaneous bilateral laparoscopic adrenalectomy. This operation is indicated in case of primary hypercortisolism caused by bilateral adrenocortical hyperfunction, Cushing's disease after failure of pituitary surgery, ectopic adrenocorticotropic hormone (ACTH) production by a tumour inaccessible for surgical intervention, and pheochromocytoma when it occurs bilaterally in case of multiple endocrine neoplasia type 2A and 2B. Different laparoscopic approaches have been described to perform this operation, such as the "anterior" approach (transperitoneal), the "lateral" approach (transperitoneal and retroperitoneal), and the "posterior" approach (retroperitoneal). We report a case of bilateral laparoscopic adrenalectomy in a 33 years old female affected with bilateral pheochromocytoma due to multiple endocrine neoplasia type 2A treated with a bilateral transperitoneal laparoscopic adrenalectomy and disease free after 18 months follow-up.     

Synchronous bilateral adrenalectomy for adrenocorticotropic-dependent Cushing's syndrome.

Select patients with ACTH-dependent Cushing's syndrome, such as patients with persistent Cushing's disease after failed hypophysectomy or patients with ectopic ACTH production, may require bilateral adrenalectomy. Laparoscopic bilateral adrenalectomy has been described, offering definitive treatment with reduced morbidity compared with open techniques. We report on the performance of synchronous bilateral adrenalectomy treated using the da Vinci robot (Intuitive Surgical, Sunnyvale, CA). To our knowledge, the usage of this minimally invasive approach for this operation has yet to be reported in literature. The details of the case and a brief review of the literature are described herein.     

Hyperadrenalism in childhood and adolescence.

Hyperadrenalism in childhood and adolescence has unique features that influence diagnosis and management. We reviewed our experience with 18 patients, ranging in age from 18 months to 18 years. Nine had bilateral adrenal hyperplasia, eight had adrenal neoplasms, and one had micronodular hyperplasia. Patients with congenital adrenal hyperplasia and hyperaldosteronism were excluded. Six patients with Cushing's disease diagnosed in earlier years were treated by total adrenalectomy and recently two patients underwent transsphenoidal removal of pituitary tumors. Bilateral adrenalectomy was carried out in one patient with micronodular hyperplasia and in a second because of elevated adrenocorticotrophic hormone (ACTH) levels from an undefined source. Eight patients had adrenal neoplasms, including five adenomas and three carcinomas. We found no reliable criteria to differentiate before surgery between adrenal adenomas and adrenal carcinomas. The most recognizable characteristic of malignancy was tumor size, specifically weight greater than 75 gms. Of the three patients with adrenal carcinoma, one expired 20 months after adrenalectomy and 8 months after receiving palliative partial hepatectomy for liver metastasis. Two patients are well with normal growth and development at 11 and 20 years following adrenalectomy. With the exception of one patient who died 6 years after surgery from a glioblastoma multiforme, all patients with adrenal adenomas are well. Eight patients underwent bilateral adrenalectomy for hypercortisolism. Five of the six who have reached their adult stature are significantly stunted. Four of six patients with Cushing's disease, treated by total adrenalectomy, have developed Nelson's syndrome at 2, 6, 10, and 12 years after surgery. Of the two patients undergoing transsphenoidal surgery, one had recurrent disease at 2 years and was treated by pituitary irradiation with recovery. The patients undergoing adrenalectomy for micronodular hyperplasia and ectopic ACTH are well at 2 and 4 years, respectively. Cushing's disease in children and adolescents is best treated by transsphenoidal removal of the pituitary adenoma. Adrenalectomy, once the most accepted approach, plays a secondary role and is indicated primarily in micronodular adrenal hyperplasia, in patients with ectopic ACTH production of an undefined source, and in recurrent Cushing's disease following prior pituitary irradiation. The high incidence of Nelson's syndrome in children treated by adrenalectomy mandates that patients at risk be monitored lifelong for the progression of a pituitary tumor.(ABSTRACT TRUNCATED AT 400 WORDS)