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.     

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.     

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.     

Unrecognized adrenal insufficiency in patients undergoing laparoscopic adrenalectomy

Background  Subclinical Cushing’s syndrome (SCS) is a well-described phenomenon where abnormalities of the hypothalamic–pituitary–adrenal axis exist in the absence of overt signs and symptoms of classic Cushing’s syndrome. While this has been shown to exist in 5–20% of patients with adrenal lesions, no standardized biochemical regimen exists to screen for SCS. Consequently, many of these patients may not be diagnosed prior to adrenalectomy with the risk of postoperative adrenal insufficiency. We began checking morning (a.m.) serum cortisol levels on postoperative day 1 (POD1) following unilateral adrenalectomy for nonfunctioning adrenal lesions to determine the incidence of unrecognized adrenal insufficiency (AI) in these patients. Methods  One hundred and five patients undergoing adrenalectomy at a tertiary care center from 1999 to 2007 were retrospectively evaluated. Patients with Cushing’s syndrome, conditions associate with bilateral disease, and those receiving perioperative steroids were excluded, leaving 41 patients for analysis. A.m. serum cortisol levels were obtained in all patients POD1. Multiple factors were analyzed as possible predictors of AI. Analysis of variance (ANOVA), t-test, and chi-square test were used to determine statistical significance. Results  The 41 patients’ diagnoses included 13 pheochromocytomas, 15 nonsecreting adenomas, 5 aldosteronomas, 5 metastatic lesions, 1 adrenocortical carcinoma, and 2 other benign lesions. Three groups were identified based on POD1, a.m. cortisol levels: sufficient (>10 μg/dl; n = 25, 61%), low-normal (3.4–10 μg/dl; n = 7, 17%), and insufficient (<3.4 μg/dl; n = 9, 22%). Tumor size and presence of diabetes, hypertension, and obesity were predictive of postoperative AI (p < 0.05). Conclusions  AI after unilateral adrenalectomy without evidence of cortisol hypersecretion on preoperative screening was present in a significant number of patients in our series. Patients with diabetes, hypertension, obesity, and larger tumors may be at higher risk for postoperative AI. More thorough screening for cortisol hypersecretion may be warranted in patients with these characteristics, and obtaining routine postoperative cortisol levels may avoid potentially dangerous unrecognized adrenal insufficiency following adrenalectomy.     

Laparoscopic adrenalectomy: lessons learned from an initial series of 100 patients

Background Laparoscopic adrenalectomy is considered the gold standard for the surgical treatment of adrenal disorders in most centers. This study analyzes the authors’ experience using the lateral intraperitoneal approach with the first 100 patients. In addition to analyzing the authors’ experience, this article aims to contrast it with some published series as an internal quality control. Methods In a 10-year period, 138 laparoscopic adrenalectomies were performed for 100 patients. Demographics, surgical results, complications, and long-term outcomes were analyzed. Results The participants comprised 69 women and 31 men with a mean age of 37 years. The procedures included 24 right, 38 left, and 38 bilateral adrenalectomies. The indications for surgery were Cushing’s disease for 33 patients, pheochromocytoma (4 bilateral) for 23 patients, Cushing’s syndrome for 18 patients, Conn’s syndrome for 16 patients, and incidentaloma for 10 patients. Five procedures were converted to open surgery. Two patients with pheochromocytoma required intraoperative blood transfusion. The mean operative time was 174 min for unilateral adrenalectomies and 302 min for the bilateral procedures. The mean hospital stay was 5 days. Surgical morbidity included an abdominal wall hematoma, a small pneumothorax, and intraabominal bleeding in one patient that required reexploration. There were three operative mortalities not related to the technique. The long-term results showed control of hypercortisolism in all the patients with Cushing’s disease and 82% of the patients with pheochromocytoma. Most of the patients with Conn’s syndrome (91.4%) became normotensive after surgery. Conclusions Laparoscopic adrenalectomy is safe and effective. The complications are mild, and mortality is related more to the patient’s condition than to the surgical technique.     

Adrenalectomy for Treatment of Cushing Syndrome: Results in 122 Patients and Long-Term Follow-up Studies

n = 122) who underwent adrenalectomy from 1957 through 1993 were reviewed for survival and complications. Of the 122 patients, 70 had adrenocortical adenoma, 30 Cushing’s disease, 6 primary pigmented nodular adrenocortical disease (PPNAD), 7 other types of primary nodular hyperplasia, 5 adrenocortical carcinoma, and 4 ectopic ACTH syndrome. Sixty-five patients with adrenocortical adenoma are alive, and the survival rate was equal to the age-matched control population, when patients who died of the postoperative complication were excluded. Of the patients with Cushing’s disease, 20 are alive; and 10 of 16 patients (63%) who were followed-up and evaluated had skin pigmentation. Four of sixteen patients (25%) developed Nelson’s syndrome. Four PPNAD patients and five with other types of nodular hyperplasia are alive. Most of these patients underwent bilateral total adrenalectomy, but some patients remitted after unilateral adrenalectomy. All of five adrenocortical carcinoma patients and four with ectopic ACTH syndrome died within 2 years after operation. The prognosis for patients with adrenocortical adenoma after unilateral adrenalectomy is excellent, though it is important to avoid operative complications. The rapid cure of signs and symptoms of glucocorticoid excess after total adrenalectomy is ensured, and prognosis is satisfactory under careful glucocorticoid replacement, making total adrenalectomy an alternative treatment for Cushing’s disease.     

Outcome of Adrenalectomy for Cushing’s Syndrome: Experience from a Tertiary Care Center

Adrenalectomy continues to play an important role in the management of Cushing’s syndrome (CS). Untreated CS causes considerable physical and mental morbidity and mortality. However, little information is available on the effect of adrenalectomy in ameliorating functional disabilities in CS patients. Our study assesses the long-term outcome of adrenalectomy in patients with CS. This is a retrospective analysis of CS patients managed during 1990–2005 at a tertiary care center. We analyzed the clinical presentation, endocrine evaluation, and surgical management preoperatively and following adrenalectomy. The subjects were 37 patients with CS (age 24.5 ± 15 years, range 1–60 years; male:female 1.0:1.2). There were various etiologies—unilateral adrenocortical adenoma (n = 11), adrenocortical carcinoma (n = 13), pituitary ACTH-secreting adenoma with failed transsphenoidal surgery (n = 4), ectopic unidentified ACTH source (n = 7), bilateral adrenal macronodular hyperplasia (n = 1), primary pigmented nodular adrenal hyperplasia (n = 1) —for which the patients underwent adrenalectomy: unilateral (n = 22), bilateral (n = 13), or adrenonephrectomy (n = 2). Two patients died during the perioperative period owing to chest infection and sepsis. At the median follow-up of 60 months (range 6–144 months), the patients exhibit significant persistence of obesity (41%), proximal muscle weakness (44%), menstrual irregularity (8%), hypertension (31%), and insulin-dependent diabetes (29%). Hirsutism and psychological abnormalities persisted to a lesser extent. All patients had biochemical cure of CS following surgery evidenced by the 8 a.m. basal cortisol ≤ 5 μg/dl. The hypothalamic-pituitary-adrenal axis recovered as shown by normalization of the short synacthen-stimulated cortisol level (peak level ≥ 20 μg/dl) after a median follow-up of 9 months (range 6–18 months). Incomplete clinical recovery following adrenalectomy emphasizes the need of early recognition and prompt treatment of CS. Surgery for adrenocortical adenoma is safe and effective; however, survival of patients with CS due to adrenocortical carcinoma remains poor. Bilateral adrenalectomy provides early control of hypercortisolism in selected cases of unlocalized ectopic ACTH syndrome or failed transsphenoidal surgery. Even though functional recovery is incomplete after adrenalectomy, quality of life improves considerably.     

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.     

Functional Results After Endoscopic Subtotal Cortical-Sparing Adrenalectomy

Purpose: We examined the required amount of residual adrenal tissue and whether an intact adrenal vein are necessary to achieve sufficient function after endoscopic subtotal adrenalectomy. Method: Endoscopic subtotal adrenalectomy was performed in 14 patients. Two patients underwent unilateral subtotal and contralateral total adrenalectomy and another two patients underwent unilateral subtotal adrenalectomy after contralateral total adrenalectomy several years earlier. We analyzed the postoperative serum levels of cortisol and adrenocorticotropic hormone (ACTH). Patients with bilateral tumors underwent an ACTH test. Results: We had to cut the main adrenal vein in ten patients, and less than one third of the adrenal gland was left in situ in four patients. Subtotal adrenalectomy was performed unilaterally in two patients with bilateral tumors. One third of the adrenal gland was preserved in these patients, and also in the two patients with unilateral subtotal adrenalectomy after previous contralateral total adrenalectomy. The postoperative ACTH test confirmed satisfactory adrenocortical function. During the follow-up period of about 24 months no recurrent tumors have been found. Conclusion: Subtotal cortical-sparing adrenalectomy can be successfully performed laparoscopically. The venous drainage of the main adrenal vein does not seem to be crucial for sufficient adrenocortical function. We estimate that leaving about one third of the entire adrenal gland as remnant adrenal tissue will result in sufficient function. Received: March 25, 2002 / Accepted: September 3, 2002 Reprint requests to: M. Brauckhoff     

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     

Posterior Retroperitoneoscopic Adrenalectomy for Clinical and Subclinical Cushing’s Syndrome

Background  

Because of co-morbidity, adrenalectomy for adrenal Cushing’s syndrome may be associated with an increased complication rate and long operating times. In the present study we report our experience with the posterior retroperitoneoscopic adrenalectomy in a large group of patients with clinical or subclinical Cushing’s syndrome.     

Laparoscopic bilateral adrenalectomy: results for 30 consecutive cases

Background Most patients requiring bilateral adrenalectomy have adrenocorticotropin hormone (ACTH)-dependent Cushing’s syndrome. Some of these patients are severely debilitated from the chronic effects of cortisol overproduction. This study aimed to analyze the indications, safety, efficacy, and outcomes for laparoscopic bilateral adrenalectomy from the authors’ experience. Methods A retrospective review was conducted at a university tertiary referral center. Between March 1996 and August 2006, 30 consecutive patients underwent simultaneous laparoscopic bilateral adrenalectomy. The patient records were analyzed to obtain patient demographics, disease etiology, surgical approach, operating room information, postoperative complications (30 days), hospital length of stay (LOS), and follow-up information. Results The 30 participants (22 women and 8 men) had a mean age of 44 years. The indications for bilateral adrenalectomy were refractory Cushing’s disease (n = 16), occult ectopic ACTH syndrome (n = 9), and bilateral pheochromocytoma (n = 5). A mean of 53 months elapsed between onset of symptoms and adrenalectomy. Laparoscopic bilateral adrenalectomy was completed for all the patients with no intraoperative complications. Four patients (13%) experienced six complications. The mean postoperative LOS was 3.5 days (range, 1–12 days). Seven patients required a preoperative LOS, for a mean of 7.1 days (range, 1–20 days), and a postoperative LOS, for a mean of 5 days (range, 2–12 days). The 23 patients who did not require preoperative hospitalization had a mean postoperative LOS of 3 days (range, 1–7 days). All the patients received postoperative steroid replacement and appropriate follow-up assessment with an endocrinologist. At this writing, the patients with Cushing’s syndrome available for follow-up evaluation continue to receive steroid replacement, and all the pheochromocytoma patients have experienced a documented postoperative biochemical cure. Conclusions Laparoscopic bilateral adrenalectomy is safe and effective for this high-risk patient population. Although patients should be monitored closely in the postoperative period, most are discharged with glucocorticoid and mineralocorticoid replacement in a short time without complications.     

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.     

单侧肾上腺切除治疗原发性醛固酮增多症对侧肾上腺储备功能的评价分析

目的对比分析原发性醛固酮增多症(PHA)患者单侧肾上腺切除前后肾上腺皮质分泌功能变化,评估分析单侧肾上腺切除后对侧肾上腺皮质储备功能。方法选取2006年1月至2013年2月接受单侧肾上腺切除手术的42例患者的临床资料。对患者手术前后早晨空腹血皮质醇、促肾上腺皮质素(ACTH)水平、以及促肾上腺皮质激素兴奋1mg地塞米松抑制试验结果进行了有效的统计学分析。结果 42例患者均无出现肾上腺皮质功能不全。手术前后基础皮质醇水平无明显差异。然而,单侧肾上腺切除术后基础ACTH水平明显提高。促肾上腺皮质激素兴奋1mg地塞米松抑制试验的皮质醇峰值水平明显下降。术后皮质醇峰值水平约为术前82.7%(76.0%~91.4%)。结论不伴有皮质醇增多症或亚临床型皮质醇增多症的PHA患者行单侧肾上腺切除,术后血皮质醇水平可维持在正常范围之内,但肾上腺皮质储备功能出现不同程度的下降,其储备功能不低于术前80%,足以使患者术后不会出现肾上腺皮质功能不全。     

Laparoscopic Cortical-Sparing Adrenalectomy for Bilateral Pheochromocytoma

A 32-year-old female with asthma was hospitalized for pneumonia in 2/06. She underwent a CT scan of the chest which revealed an incidental finding of bilateral adrenal masses. On further questioning, she admitted to palpitations and flushing. She was normotensive. Biochemical workup was significant for elevated urinary norepinephrine and normetanephrines, and plasma catecholamine level. MIBG scan showed positive uptake in the left adrenal gland consistent with pheochromocytoma. T2 weighted MRI showed bilateral adrenal masses, left greater than right. After adequate alpha blockade with phenoxybenzamine, the patient underwent a laparoscopic left adrenalectomy. Pathology revealed a 3.5 cm pheochromocytoma. The patient then underwent a right cortical-sparing adrenalectomy to avoid complete adrenal insufficiency and Addisonian crisis. The choice of operation was made realizing the potential for increased bleeding, which was further complicated by the patient’s Jehovah’s Witness beliefs, which prohibit transfusion of any blood products. At surgery, a small, well-circumscribed mass of the inferior right adrenal gland was found, and excised in its entirety. A postoperative ACTH-stimulation test showed appropriate cortisol response. Pathology revealed a 1.5 cm pheochromocytoma, and the patient recovered uneventfully. Cortical-sparing adrenalectomy has been reported with success rates of 65–100% in avoiding exogenous steroid dependence.^1,2 Bilateral pheochromocytoma remains the most common indication. Risks for both recurrence and malignancy require lifelong follow-up in these patients. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Early post-operative ACTH and cortisol as predictors of remission in Cushing’s disease

Summary Aim. To study the value of early (24 h) post-operative ACTH and serum cortisol as predictors of remission after transsphenoidal surgery in Cushing’s disease. Methods. We prospectively studied 44 patients who underwent transsphenoidal surgery for Cushing’s disease between 1997 and 2005. The mean follow-up period of patients after surgery was 49 months (19–102 months). The predictive value of clinical characteristics, pre-operative hormonal studies, radiological, surgical and histological findings, and post-operative hormonal studies were analysed. For the post-operative hormonal study plasma ACTH and serum cortisol were determined at 8.00 a.m. the day after surgery. Results. After surgery, Cushing’s disease remitted in 39 patients (89%) and persisted in 5 patients (11%). Three patients relapsed during the follow-up period. Only three study variables were predictive of persistence of Cushing’s disease after surgery: the non identification of the adenoma in histology (an adenoma was found in 87% of the patients in remission, and in 20% of treatment failures, p = 0.01), the early post-operative plasma ACTH (patients in remission: 2 pmol/L (1.1–10.8 pmol/L), treatment failures: 8.2 pmol/L (1.1–12 pmol/L), p = 0.019), and the early post-operative serum cortisol (patients in remission: 128.4 nmol/L (27.6–4644 nmol/L), treatment failures: 797 nmol/L (606–1037 nmol/L), p = 0.003). ROC curves indicated that plasma ACTH ≤7.55 pmol/L distinguished patients in remission from treatment failures with 80% sensitivity and 97.4% specificity, and serum cortisol ≤585 nmol/L with 100% sensitivity and 90% specificity. Conclusions. Twenty-four hours after transsesphenoidal surgery for Cushing’s disease, and without glucocorticoids replacement, patients with serum cortisol concentrations higher than 585 nmol/L, and/or plasma ACTH higher than 7.55 pmol/L, and/or those in which an adenoma is not identified in the histological study, have a high risk of treatment failure.     

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.     

Diagnosis and treatment of primary aldosteronism

Disorders of the adrenal cortex and medulla are often associated with hypertension, which can be cured surgically in many cases or may require specific and timely medical treatments. Therefore, knowledge of adrenal physiology, biochemistry, and molecular biology is essential such that an appropriate diagnostic evaluation can be conducted efficiently. The most common hypertensive disorder of the adrenal cortex is primary aldosteronism. Aldosterone-producing adenoma is the most common form of primary aldosteronism and is most likely to be cured by unilateral adrenalectomy when aldosterone production is highly autonomous from renin-angiotensin, lateralizes to one adrenal gland, and is associated with overproduction of 18-hydroxycortiocosterone and C18-methyloxygenated metabolites of cortisol. Variants of adrenal hyperplasia that share these characteristics can also be cured by unilateral adrenalectomy.