Current management of pheochromocytoma: about 50 cases

STUDY AIM: The aim of this retrospective study was to assess our experience of the laparoscopic surgery of pheochromocytoma.We report indications and results of laparoscopic and open adrenalectomy for pheochromocytoma. PATIENTS AND METHODS: Between january 1994 and may 2002, 50 patients underwent laparoscopic or open adrenalectomy for pheocromocytoma. The perioperative hemodynamic parameters were assessed for each patient. In each case, urinary metanephrine levels were measured at the second month postoperatively. The long term outcome was assessed in 44 patients. The mean follow-up was 39 months. RESULTS: Ten patients underwent open adrenalectomy: 8 patients for unilateral tumors (tumor size was > 8 cm in 7 cases) and 2 patients for bilateral tumors (1 recurrence and 1 cystic polylobed tumor). Fourty patients underwent laparoscopic adrenalectomy: in 32 cases, including 1 patient with a bilateral tumor, no conversion was performed (tumor size was < 5 cm in 29 cases). In 8 cases (20%), a conversion to an open operation was performed. The reasons to convert were bleeding and periadrenal fibrosis in 7 cases. In laparoscopic adrenalectomy group, hemodynamic troubles were not more frequent, the hospital stay was shorter and there was no recurrence. CONCLUSION: Laparoscopic adrenalectomy is the Gold standard procedure for patients with pheochromocytoma. But open adrenalectomy is sometimes indicated: tumor size > 8 cm, periadrenal fibrosis, and recurrence tumor.     

Cushing syndrome associated with prostatic tumor adrenocorticotropic hormone (ACTH) expression after maximal androgen blockade therapy

We report a patient with adenocarcinoma of the prostate, who eventually developed Cushing syndrome due to ectopic adrenocorticotropic hormone (ACTH) secretion from the tumor. At first, maximal androgen blockade (MAB) therapy was effective for the prostate carcinoma, which was positive for prostate-specific antigen (PSA) and negative for ACTH on the biopsy specimen. However, 3 years later, the patient complained of bilateral leg edema. A chest computed tomographic (CT) scan showed bilateral pleural effusion and inflammatory changes, focused on the right upper-lobe. While his PSA was not elevated, and there were no obvious tumor metastases, his serum cortisol and ACTH levels were elevated, without any evidence of lesions that could release ectopic ACTH. Two weeks later, the patient complained of dyspnea and bilateral pleural effusion, and inflammatory changes were worse. Although the patient was administered inhibitors of adrenocorticoid synthesis-metyrapone, they did not have enough clinical efficiency. Steroid pulse therapy was also administered but the patient's severe pneumonia and pleural effusion did not improve and he finally died of respiratory failure. In contrast to the initial biopsy specimen findings, on autopsy, the tumor was negative for PSA but positive for ACTH. Thus, it would appear that the tumor began to produce and release ectopic ACTH after therapy, which resulted in the development of Cushing syndrome in this patient with prostate carcinoma.     

Cervical sympathectomy affects adrenocorticotropic hormone and thyroid-stimulating hormone in rats

To examine the effects of bilateral cervical sympathectomy on the secretion of adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH), growth hormone (GH), and prolactin (PRL), 18 male rats were divided into three groups: control (Cont), sham operation (Sham), and bilateral cervical sympathectomy (Symp). All rats were kept under a normal circadian rhythm for 2 weeks. Subsequently, blood was collected and plasma ACTH as well as serum TSH, GH, and PRL levels were measured. The difference in ACTH levels between the Cont and Sham groups was not significant, but ACTH levels in the Symp group were significantly higher than those in the other groups. The difference in TSH levels between the Cont and Sham groups was also not significant, but TSH levels in the Symp group were significantly lower than those in the Cont group. There were no statistically significant differences in GH and PRL levels among these groups. The present results suggest that cervical sympathectomy in the rat increases ACTH secretion and decreases TSH secretion in the pituitary. These effects seem to be due to a mildly increased secretion of melatonin in the pineal body that probably in turn increases corticotropin-releasing factor (CRF) secretion and decreases thyrotropin-releasing hormone (TRH) secretion in the hypothalamus. Extrapolation of these findings to humans suggests that longterm and repeated stellate ganglion block would affect the pituitary secretions of ACTH and TSH.     

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.     

Critical size of residual adrenal tissue and recovery from impaired early postoperative adrenocortical function after subtotal bilateral adrenalectomy

BACKGROUND: Subtotal bilateral adrenalectomy may preserve adrenocortical function. Little is known about the early postoperative function of the adrenal remnant. METHOD: In 10 patients with bilateral adrenal tumors (pheochromocytomas, adrenocortical nodular hyperplasia, and adrenal metastases), plasma adrenocorticotropic hormone (ACTH), serum cortisol, and maximal cortisol liberation were examined with an ACTH test after subtotal bilateral adrenalectomy, which left 15% to 30% of adrenal tissue in situ. RESULTS: In the early postoperative period, all patients had normal basal serum cortisol levels (mean, 415+/-208 nmol/L; normal morning range, 138-690 nmol/L) but pathologically increased plasma ACTH levels (mean, 55+/-42 pmol/mL; normal, <10.1 pmol/L). In 6 patients, a pathologic ACTH test result was observed. During follow-up (mean, 11.3+/-7.6 months), all patients were found to have a normal ACTH test result. None of the patients required steroid supplementation. However, in patients with both familial pheochromocytoma and impaired adrenocortical function during the early postoperative period, the maximal increase of serum cortisol after ACTH stimulation was significantly reduced (mean, 301+/-86.8 nmol/L) compared with control subjects (mean, 490+/-132.6 nmol/L; P=.019). CONCLUSION: After subtotal bilateral adrenalectomy left 15% to 30% of adrenal tissue in situ, functional recovery could be observed in all patients. However, subclinical impairment of the adrenocortical function with questionable clinical significance has to be considered in some of the patients. Especially during the early postoperative period, careful observation of the patients without exogenous steroid administration is required.     

成龄大鼠肾上腺同种异体肾被膜下种植的实验研究

将１２０只成龄雌性Ｗｉｓｔａｒ大鼠双侧肾上腺全切除后，将异体肾上腺组织块种植于肾被膜下（ＳＲＣ）。结果显示：种植组织可存活及再生。单纯种植组术后早期种植组织有淋巴细胞浸润，而应用环孢素Ａ（ＣｓＡ）能抑制排斥反应，再联用ＡＣＴＨ则可促进移植体再生。一定程度再生后停用ＣｓＡ，种植体可长期存活，并发挥正常的分泌功能，其效果与吻合血管的异体肾上腺移植相同。本法技术简单，取材方便，为临床肾上腺同种异体移植提供一简便有效的方法。     

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.     

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     

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。     

Adrenal failure due to bilateral adrenal metastasis of rectal cancer: A case report

IntroductionIt is rare for a patient to present with adrenal insufficiency secondary to bilateral adrenal metastases from a malignant colorectal tumor.Case presentationAn 82-year-old Japanese man presented to our hospital with high fever and malaise. He was receiving oral chemotherapy for the treatment of rectal cancer with multiple metastases. Computed tomography showed new bilateral adrenal gland metastases. A rapid adrenocorticotropic hormone (ACTH) test showed adrenal insufficiency. Treatment with hydrocortisone provided immediate symptom improvement.DiscussionAdrenal insufficiency secondary to bilateral adrenal metastases from rectal cancer is rare. A rapid ACTH test is useful to diagnose adrenal insufficiency.ConclusionThe incidence of adrenal insufficiency may be underestimated in patients with multiple metastasis. Appropriate therapy with adrenal corticosteroid hormone supplementation may lead to a significant improvement in the patient’s symptoms and quality of life.     

Impact of laparoscopy on the management of adrenal diseases. A retrospective study of 220 patients

OBJECTIVE: Soon after its introduction in 1992, laparoscopic adrenalectomy became the gold standard in the surgical management of most adrenal tumors. The aim of this study was to assess the influence of laparoscopy on surgical indications. PATIENTS AND METHODS: Between 1994 and 2003, 220 adrenalectomies were performed, 179 among them by a laparoscopic approach. There were 137 females and 83 males. The mean age was 53 years (range 15-83 years). RESULTS: The indications of adrenalectomy were: Cushing syndrome 18%, pheochromocytoma 31%, Conn syndrome 16%, incidentaloma 21%, and malignant tumours 13%. Laparoscopic approach was performed in 81% of the cases and the conversion rate was 11%. There were 3 postoperative deaths (2 after laparoscopy). The mean hospital stay was 7.6 days in the laparoscopic group, and 13.6 days in the open surgery group. CONCLUSIONS: This study is consistent with the findings of the literature supporting that there are no indications for the open procedure in case of small benign lesions. The video-asisted adrenalectomy had not changed the management of the adrenal incidentaloma. Today, the laparoscopic approach seems to be adapted also for malignant disease.     

Presentation of a medullary endocrine neoplasia 2A kindred with Cushing's syndrome

Although medullary thyroid cancer (MTC) can produce adrenocorticotropic hormone (ACTH) in up to 40 per cent of cases as determined by immunohistochemistry, clinical hypercortisolism is rarely seen. We report a medullary endocrine neoplasia 2A (MEN 2A) kindred whose proband case presented with Cushing's syndrome (CS). This 51-year-old woman presented with debilitating weakness, exertional dyspnea, 50 pound weight gain, moon facies, worsening hypertension, striae, and hirsutism. A comprehensive evaluation diagnosed ectopic ACTH production from unresectable metastatic MTC to the liver. Genetic testing revealed a germline RET proto-oncogene mutation at codon 609. Further genetic testing identified six family members with the same mutation. The patient underwent palliative bilateral laparoscopic adrenalectomies with significant improvement in major comorbidities. Overall CS resulting from ectopic ACTH overproduction by MTC is rare, occurring in 0.6 per cent of all patients with medullary thyroid carcinoma. About 50 cases have been previously reported in the literature, but only three in families with MEN 2A. We describe the first case of a MEN 2A kindred presenting with CS from ectopic ACTH production by metastatic medullary thyroid carcinoma. We advocate consideration of early bilateral laparoscopic adrenalectomies in patients with symptomatic hypercortisolism from unresectable metastatic medullary thyroid carcinoma.     

Usefulness of multiple-site venous sampling in the treatment of adrenocorticotropic hormone-producing pituitary adenomas

BACKGROUND: While venous sampling for adrenocorticotropic hormone (ACTH) may be the most accurate way to establish a diagnosis of Cushing's disease, its usefulness for lateralization of adenomas is limited even in combination with simultaneous bilateral sampling methods. Therefore, to increase accuracy in predicting the lateralization of adenomas, we performed a trial in which we carried out simultaneous venous sampling from multiple sites of the cavernous sinus (CS) and inferior petrosal sinus (IPS). Here we report on the usefulness of our method in the correct tumor lateralization in patients with Cushing's disease. METHODS: Eighteen patients with Cushing's disease underwent simultaneous bilateral ACTH sampling. The samples were obtained from the anterior, middle, and posterior CS and the IPS. CS sampling after stimulation with corticotropin-releasing hormone (CRH) was also performed. The central-to-peripheral ACTH ratio (c/p ratio) was calculated using sampling data from each site; the lateralizing gradients (right versus left) were calculated using the c/p ratio. Instead of the conventional method where the lateralization gradient is based on sampling data from a single site, we used the distribution of the c/p ratio determined from multiple-site data. RESULTS: There was no significant difference in the mean lateralization gradient obtained from each set of sampling data (p > 0.05). With the conventional method, correct tumor lateralization was obtained in 50% of tumors in the IPS; 72.2 to 77.8% of tumors in the CS; and 77.8% of tumors in the middle CS after CRH. Our method using ACTH contour analysis based on multiple sites produced correct results in all 18 of the patients examined. The difference in correct calls between the conventional method and our method was significant (p < 0.05). CONCLUSIONS: Multiple-site sampling of ACTH is valuable for lateralizing the adenoma in patients with Cushing's disease; it produces more correct results than does single venous sampling.     

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%).     

迟发型先天性肾上腺皮质增生伴睾丸肾上腺残余肿瘤临床分析(附1例报告)

目的:分析迟发型先天性肾上腺皮质增生(congenital adrenal hyperplasia,CAH)伴睾丸肾上腺残余肿瘤(testicular adrenal rest tumors,TART)的临床诊疗方法。方法:对2009年本院男科门诊收治的1例迟发型CAH伴TART的患者临床资料进行回顾性分析。结果:患者年龄15岁,身材矮小,皮肤黝黑,牙龈及外生殖器等皮肤色素沉着,第二性征呈成人型,双侧睾丸触及不规则肿大质硬结节。血促肾上腺皮质激素(ACTH)、尿17-酮类固醇(17-KS)、硫酸脱氢表雄酮(DHEA-S)、血孕酮(PRGE)明显升高。卵泡刺激素(FSH)、黄体生成素(LH)、皮质醇(CO)明显降低。小剂量地塞米松抑制试验ACTH、DHEA-S降至正常水平。影像学检查双侧肾上腺软组织密度影,右侧较为明显。双侧睾丸见不规则增大,左侧较为明显,信号不均,内见分隔,周围见液性信号环绕。病理学示嗜酸性胞质呈多角形或圆形,间质样细胞条索状排列,胞质内含有脂色素,免疫组化示排除睾丸间质细胞瘤。地塞米松替代治疗3个月后临床症状逐渐改善,睾丸结节左侧明显缩小,右侧消失,肾上腺增生结节9个月时消失。结论:根据以上临床表现和辅助检查,诊断为迟发型CAH伴TART,因肾上腺皮质功能不足致ACTH的持续大量分泌,最终导致肾上腺皮质增生和TART发生,通过足量的地塞米松替代治疗后TART缩小或消失,肾上腺增生结节消失,临床症状改善,各项实验室指标恢复正常。     

Lessons learned from 274 laparoscopic adrenalectomies

AIMS: To define the role of minimally invasive video-assisted surgery in the surgical management of adrenal disease and discuss the respective indications of the trans and retroperitoneal video assisted approaches. MATERIALS AND METHODS: During the last 8 years (1994-2001), 330 adrenalectomies were performed in 305 patients: 274 (83%) laparoscopic approaches and 56 (17%) open approach. Open surgery was reserved for patients presenting with large or malignant tumours (29 cases), multiple and/or extraadrenal phaeochromocytomas (13 cases), previous intraabdominal intestinal surgery (10 cases), and in those requiring concomitant intraabdominal surgery (4 cases). Laparoscopic adrenalectomy was performed using the lateral transperitoneal approach for 89 Conn's syndrome, 67 Cushing's syndrome, 2 virilizing tumours, 51 phaeochromocytomas and 65 non secretory tumours greater than 4 cm in diameter. Nineteen patients underwent bilateral adrenalectomy. RESULTS: There were no deaths. Twenty patients (7.3%) had a complication. Eleven cases required open conversion (4%) because of difficulties with dissection (8 cases), preoperative suspicion of malignancy (2 cases), and one pneumothorax. The average size of tumours was 34 mm (7-110 mm). There were 18 malignant tumours (6.5%): 8 adrenocortical carcinomas, 1 leiomyosarcoma, and 9 metastases. All patients with hormonally secreting tumours were cured of their endocrinopathy. There was 1 death secondary to hepatic metastases in a patient with an adrenocortical carcinoma. CONCLUSION: Most adrenal tumours are suitable for video assisted excision. The only absolute contraindication is an invasive carcinoma requiring an extended excision. The lateral, transperitoneal approach is the most suitable for tumours greater than 5-6 cm in diameter. Both the transperitoneal or retroperitoneal approaches are suitable for smaller tumours depending on operator choice and experience. However in the presence of a large right lobe of liver or previous intraabdominal surgery the retroperitoneal approach may be preferable.     

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。     

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     

Successful treatment of Cushing disease with the sphenoid sinus of conchal type: usefulness of inferior petrosal sinus sampling and STEALTH navigation system: a case report

We report a case of Cushing disease whose microadenoma was not identified by MRI with dynamic study and whose sphenoid sinus was conchal in type. Venous sampling test in the bilateral inferior petrosal sinus demonstrated ACTH hypersecretion on the right side. The patient underwent transsphenoidal surgery. A poorly pneumatized sphenoid sinus was drilled effectively, guided by the STEALTH navigation system. After the first operation, the patient's serum ACTH concentration was still high, suggesting that tumor removal was incomplete. Then she underwent the second operation and the residual tumor was completely removed with the assistance of the STEALTH navigation system again. Postoperatively, her serum ACTH concentration dropped below 5 pg/ml. In this case, inferior petrosal sinus sampling was extremely helpful for the diagnosis of Cushing disease, and operations were able to be performed safely using the neuronavigation system in drilling of the incompletely pneumatized sphenoid sinus.     

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.