Adrenocorticotropic Hormone-dependent Cushing's Syndrome Research Articles

THU531 A Case Of Cushing Syndrome Due To Ectopic ACTH Secreting Lung Neuroendocrine Tumor Treated With Cryoablation

Abstract Disclosure: S. Paknikar: None. J. McHugh: None. K. Rachmasari: None. I. Bancos: None. Introduction: Ectopic secretion of adrenocorticotropic hormone (ACTH) is a rare and severe form of Cushing Syndrome (CS) that requires an urgent specialized approach for diagnosis and management. Clinical Case: A 50-year-old woman was referred to endocrinology due to concern for CS. Over the previous year she had developed progressive weakness, edema, weight gain with abdominal fat redistribution, hypertension, and multiple compression fractures. On exam, she had a profound proximal myopathy, reported 10/10 pain along the spine, and was hypertensive (170/105 mm Hg) with normal other vitals. The patient's sodium was 141mmol/L [reference range 135-145 mmol/ L], potassium 4.1 mmol/L [3.6-5.2 mmol/L] while on spironolactone, and creatinine was 0.57 mg/dL [0.59-1.04 mg/dL]. Due to the severity of her symptoms, she was admitted to the hospital. Hormonal work up was consistent with an ACTH dependent CS: ACTH was 121pg/mL [7.2-63 pg/mL] and 24-hour urine cortisol was 645 mcg/24 hr [3.5-45 mcg/24 hr]. Workup for ACTH secreting tumor included a pituitary MRI which showed a 3 mm area of delayed enhancement in the pituitary gland, felt to be artifactual. A chest computed tomography (CT) showed a 1.3 cm low-density nodule in the right upper lobe concerning for a neuroendocrine tumor. A Gallium-68 DOTATATE Positron emission tomography/CT scan was performed and demonstrated that the nodule was highly DOTATATE avid (Krenning score of 3, scale of 0-4). These findings supported a diagnosis of ectopic CS caused by an ACTH secreting lung neuroendocrine tumor. Management of ectopic CS was twofold: addressing the underlying ACTH secreting lesion and urgent treatment of hypercortisolism and its consequences. In this case, this was achieved with CT-guided cryoablation of the right upper lobe nodule. The ACTH levels were monitored postoperatively, and when decreased (9.3pg/mL [ 7.2-63 pg/mL], glucocorticoid replacement therapy was initiated. As a result of prolonged hypercortisolism, the patient experienced multiple compression fractures and debility. Physical therapy was recommended for recovery from the compression fractures as well as proximal myopathy. Enoxaparin was initiated due to risk of venous thromboembolism from CS. From an osteoporosis standpoint, she was continued on calcium and vitamin-D supplementation and treated with weekly alendronate. Her hypertension improved allowing medication reduction. Postoperatively, she received education on adrenal insufficiency and biochemical monitoring for adrenal insufficiency recovery was implemented. Conclusion: Ectopic ACTH secreting tumors represent a rare cause of CS and effective management requires consideration of medical comorbidities to determine the approach: surgical, cryoablative, or medical therapy alone, mitigation of Cushing syndrome sequelae, and prompt recognition and treatment of glucocorticoid withdrawal. Presentation: Thursday, June 15, 2023

Bilateral inferior petrosal sinus sampling: Procedural data from a German single-center study.

To assess radiation exposure and diagnostic performance of bilateral inferior petrosal sinus sampling for the distinction of pituitary and ectopic adrenocorticotropin-dependent Cushing's syndrome. Procedural data of bilateral inferior petrosal sinus procedures were retrospectively evaluated. The analysis included the patients' clinical and demographic data, procedural radiation exposure, and complication rates, sampling results, clinical course of the patients, and calculation of diagnostic performance data. The cases of 46 patients diagnosed with adrenocorticotropin-dependent Cushing's syndrome were evaluated. Bilateral inferior petrosal sinus sampling was successfully performed in 97.8 % of the cases. The overall median procedure-related fluoroscopy time was 7.8 min. (range 3.2-36.2 min.), and the median procedural dose area product was 11.9 Gy*cm2 (range 2.1-73.7 Gy*cm2). Radiation doses due to digital subtraction angiography series for visualization of the inferior petrosal sinus were 3.6 Gy*cm2 (range 1.0-18.1 Gy*cm2). Radiation doses due to fluoroscopy had a higher impact on the overall radiation exposure and were significantly influenced by the patients' habitus. The sensitivity, specificity, and positive and negative predictive values were 84 %, 100 %, 100 %, and 72 % before stimulation with corticotropin-releasing hormone, and 97 %, 100 %, 100 %, and 93 % after stimulation. Concordance between magnetic resonance imaging studies and bilateral inferior petrosal sinus sampling results was only found in 35.6 % of the cases. The periprocedural complication rate was 2.2 %, with one patient experiencing vasovagal syncope during catheterization. Bilateral inferior petrosal sinus sampling is a safe procedure with high technical success rates und excellent diagnostic performance. The procedure-related radiation exposure shows large variations and depends on the complexity of cannulation as well as the patients' habitus. Fluoroscopy accounted for the largest proportion of radiation exposure. Acquisition of digital subtraction angiography series for the verification of correct catheter placement appears justified. · Bilateral inferior petrosal sinus sampling with CRH stimulation provides high diagnostic performance in the distinction of pituitary and ectopic Cushing's syndrome.. · The associated radiation exposure is not negligible and is significantly influenced by the use of fluoroscopy and the patients' habitus.. · Digital subtraction angiography contributes less to the overall radiation dose and appears justified for the verification of correct catheter placement.. · Augustin A, Detomas M, Hartung V et al. Bilateral inferior petrosal sinus sampling: Procedural data from a German single-center study. Fortschr Röntgenstr 2023; 195: 1009 - 1017.

Coexistence of adrenocorticotropic hormone-dependent Cushing's syndrome and papillary thyroid carcinoma

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Bilateral Inferior Petrosal Sinus Sampling and Utility of Maximum Stimulated Petrosal Sinus ACTH to Baseline ACTH ratio in Adrenocorticotropin-dependent Cushing's Syndrome

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Limited Invasive Protocol: Optimizing Diagnostic Modalities in Corticotropin Mediated Cushing Syndrome

BackgroundTo limit the role of bilateral inferior petrosal sinus sampling (BIPSS) in distinguishing between Cushing disease (CD) and ectopic Cushing syndrome (ECS), recent reports have proposed a noninvasive approach based on a combination of biochemical testing and radiological imaging as an alternative to the conventional invasive strategy (CIS). However, this strategy requires further validation. The current study aimed to evaluate 2 limited invasive protocols (LIP-1 and LIP-2) in limiting the role of BIPSS while maintaining a diagnostic accuracy similar to that of CIS. MethodsThis was a single-center study conducted on individuals with corticotropin-dependent Cushing syndrome. The LIPs were based on performing high-dose dexamethasone suppression (>50% cut-off in first [LIP-1] and >80% in second [LIP-2]) and magnetic resonance imaging of the sella in all individuals and selective use of computed tomography of the chest and abdomen before BIPSS. These LIPs were evaluated for limiting the use of BIPSS, their accuracy, and cost in comparison to CIS. ResultsOf the 206 individuals, 114 (97 of CD and 21 of ECS) were eligible for the current study. Using LIP-1, LIP-2, and CIS, BIPSS could have been avoided in 62.3%, 35.9%, and 25.4% of individuals, respectively. The positive predictive value for CD using LIP-1 and LIP-2 was 98.9% and 100%, respectively. The cost per patient evaluated using LIP-1, LIP-2, and CIS was $602.21, $966.81, and $1107.78, respectively. ConclusionLIPs represent an equally accurate, less invasive, and more cost-effective alternative to the CIS for distinguishing between CD and ECS.

Mifepristone in the treatment of an elderly patient with adrenocorticotropic hormone-dependent Cushing syndrome

促肾上腺皮质激素（ACTH）依赖性库欣综合征（Cushing syndrome，CS）首选手术治疗。但当定位不明确或无法耐受手术时，药物治疗是重要的备选方案。本例为88岁高龄ACTH依赖性CS 患者，经米非司酮治疗后该患者CS相关症状及体征改善，降压药物减量，降糖药物停用，血钾稳定。提示对于高龄中重度CS患者，如果手术不成功或不能耐受手术，米非司酮是一种可考虑的治疗选择。.

Simultaneous pituitary and adrenal adenomas in a patient with non ACTH dependent Cushing syndrome; a case report with literature review.

IntroductionCushing syndrome is a rare disorder with a variety of underlying etiologies, that can be exogenous or endogenous (adrenocorticotropic hormone (ACTH)-dependent or ACTH-independent). The current study aims to report a case of ACTH-independent Cushing syndrome with adrenal adenoma and nonfunctioning pituitary adenoma.Case reportA 37–year–old female presented with amenorrhea for the last year, associated with weight gain. She had a moon face, buffalo hump, and central obesity. A 24-hour urine collection for cortisol was performed, revealed elevated cortisol. Cortisol level was non-suppressed after administering dexamethasone. MRI of the pituitary region revealed a pituitary microadenoma, and the CT scan of the abdomen with adrenal protocol revealed a left adrenal adenoma.DiscussionEarly diagnosis may be postponed due to the variety of clinical presentations and the referral of the patient to different subspecialists based on their dominant symptoms (gynecological, dermatological, cardiovascular, psychiatric); it is, therefore, critical to consider the entire clinical presentation for correct diagnosis.ConclusionDue to the diversity in the presentation of CS, an accurate clinical, physical and endocrine examination is always recommended.

Abstract 160: Single-center experience with Bilateral Inferior Petrosal Sinus Sampling (BIPSS) in adrenocorticotropin-dependent Cushing's syndrome

Abstract 160: Single-center experience with Bilateral Inferior Petrosal Sinus Sampling (BIPSS) in adrenocorticotropin-dependent Cushing's syndrome

Development and validation of a novel index for the differential diagnosis of corticotropin-dependent Cushing syndrome.

To develop an index for the differential diagnosis of corticotropin-dependent Cushing syndrome (CS). The development cohort included 112 consecutive patients with clinicopathologically confirmed corticotropin-dependent CS at the Department of Endocrinology and Metabolism, Tianjin Medical University General Hospital, from December 2004 to May 2020, and data of 126 patients from studies published from 2016 to August 2020, identified through search in PubMed, Embase and the Cochrane Library, was extracted for external validation. The index was calculated as the product of plasma adrenocorticotropic hormone (ACTH, pmol/L) and urinary free cortisol (UFC, nmol/24h) divided by 10,000. The discriminative ability was tested using receiver operating characteristics (ROC) curve analysis. In development cohort, area under curve of ROC analysis of the ACTH-UFC index in identifying Cushing disease (CD) was 0.977. The diagnostic accuracy of ACTH-UFC index ≤ 11 was comparable to that of 48h 8mg/d high-dose dexamethasone test (HDDST) in identifying CD, with sensitivity, specificity, positive and negative likelihood ratios of 96.6%, 87.5%, 7.73, and 0.04, respectively. The sensitivity of ACTH-UFC index ≤ 11 in parallel combination with pituitary magnetic resonance imaging (MRI) was 100% for identifying CD. The performance of the ACTH-UFC index in parallel or serial combination with pituitary MRI was similar in the validation cohort. ACTH-UFC index provides a rapid, convenient and non-invasive adjunctive approach for the differential diagnosis of corticotropin-dependent CS, with no risk of aggravating metabolic disturbances. Investigations for ectopic causes of corticotropin-dependent CS should be performed with ACTH-UFC index > 11 and negative contrasted pituitary MRI.

Pituitary magnetic resonance imaging vs. bilateral inferior petrosal sinus sampling: comparison between non-invasive and invasive diagnostic techniques for Cushing's disease-a narrative review.

Cushing's syndrome is a pathological clinical condition caused by an exposure of elevated cortisol levels over a long period of time. It is therefore essential to establish what the cause of hypercortisolism is. In most cases (about 80%) the pathological process is due to adrenocorticotropic hormone (ACTH), while in a minor part of the cases (about 20%) the cause is represented by a pathology of the adrenal glands and therefore not related to ACTH. Most patients with ACTH dependent Cushing's syndrome have a pituitary microadenoma; in the remaining cases (30%), the high level of cortisol is linked to an ectopic secretion of ACTH. Surgical removal of the pituitary adenoma represents the treatment of choice in Cushing's disease (CD) patients; it is therefore necessary to identify and precisely locate the pituitary tumour responsible for the secretion of ACTH. Adequate diagnostic information is very often, even with magnetic resonance imaging (MRI), and in these cases we rely on bilateral inferior petrosal sinuses sampling (BIPSS). This procedure is considered the gold standard method for the diagnosis, but like any other diagnostic method it is not free from erroneous results such as false positives or false negatives.

Percutaneous Bilateral Adrenal Radiofrequency Ablation in Severe Adrenocorticotropic Hormone-dependent Cushing Syndrome.

Objectives:The objectives of the study were to evaluate adrenal radiofrequency ablation (RFA) as a method of treatment in patients with severe adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome, among whom bilateral adrenalectomy is not a suitable option.Material and Methods:Five patients with ACTH-dependent Cushing syndrome underwent RFA of both adrenal glands. Four of them presented with Cushing disease unsuccessfully treated with pituitary surgery and medical therapy, while one patient had ACTH-dependent Cushing syndrome due to pancreatic endocrine tumor with liver metastases. All patients were disqualified from adrenalectomy due to morbid obesity or lack of consent.Results:A technical success was obtained in all cases, with only one re-intervention necessitated by a cooling effect of the inferior vena cava. Despite pre-procedural adrenergic blockade, severe hypertension was noted during the procedure in three cases, this being treated immediately using direct-acting vasodilators. No complications occurred otherwise. In all cases, significant improvement of clinical symptoms was observed, as well as marked decreases in levels of serum cortisol, free urine cortisol, and dehydroepiandrosterone sulfate.Conclusion:Bilateral RFA under CT-guidance is technically feasible and clinical improvement can be achieved using the method. In patients disqualified from adrenal surgery, RFA might be considered as an alternative method of ACTH-dependent Cushing syndrome treatment.

Bilateral Inferior Petrosal Sinus Sampling in Corticotropin-Dependent Cushing's Syndrome: A Single Center Experience from Western India.

Objective:Efficacy of bilateral inferior petrosal sinus sampling (BIPSS) in corticotropin-dependent Cushing's syndrome (CS) for localization and lateralization of excess adrenocorticotropic hormone (ACTH) source, as compared to high-dose dexamethasone suppression test (HDDST) and magnetic resonance imaging (MRI) pituitary, respectively.Methodology:Thirteen patients with clinically and biochemically confirmed CS underwent HDDST, MRI pituitary, and BIPSS by an experienced team of intervention neurologist, neurosurgeon, and endocrinologist using percutaneous femoral vein approach.Results:Of 13 patients (11 adults and two children) who underwent BIPSS, raised central to peripheral ACTH ratio was achieved in 12 cases, remaining one case being ectopic ACTH secretion (EAS). However, inter IPS gradient >1.4 was achieved in 11 (91.6%) of 12 Cushing's disease (CD) cases before vasopressin stimulation; and in 9 (75%) of 12 CD cases after vasopressin stimulation (P-value 0.583). HDDST suppression of more than 50% was present in only ten cases with CD, falsely negating CD in two cases (16.6%), sensitivity 83.3% and specificity 100%. MRI sella demonstrated pituitary microadenoma in 12 cases and macroadenoma in one case. Lateralization by BIPSS and MRI was concordant in 7 (58.3%) out of 12 cases with CD, with rate of remission after transsphenoidal surgery being higher in patients with concordant lateralization by BIPSS and MRI.Conclusions:BIPSS is an important investigation to distinguish CD and EAS. BIPSS was superior to HDDST for confirming the source of excess ACTH. Our findings favor the use of BIPSS for localization and pituitary MRI for lateralization of microadenoma.

Value of fluorine-18-fluorodeoxyglucose PET/CT in localizing the primary lesion in adrenocorticotropic hormone-dependent Cushing syndrome.

This study aimed to present a proper understanding of the fluorine-18-fluorodeoxyglucose (F-FDG) PET/computed tomography (CT) value in localizing the primary lesion in adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome in a relatively large case cohort. This retrospective study included 47 patients with ACTH-dependent Cushing syndrome, who underwent an F-FDG PET/CT examination in our hospital from November 2010 to January 2018. The serum cortisol, 24 h urine cortisol, serum ACTH, and high-dose dexamethasone suppression test were measured in all the patients. Bilateral inferior petrosal sinus sampling was performed in 28/47 patients. Pituitary magnetic resonance imaging and whole-body F-FDG PET/CT were performed in all patients. Serum ACTH at 8 a.m. higher than 161.8 pg/ml as the cut-off value showed the best diagnostic accuracy (73.9%) for the successful localization of the primary lesions. In Cushing disease patients, the pituitary maximum standardized uptake value F-FDG PET/CT (n=11) did not show significant differences compared with that in ectopic ACTH syndrome (EAS) patients (n=20). In terms of EAS, the descending order of maximum standardized uptake value was 13.2±8.3 (4.8-26.4) in pancreatic neuroendocrine tumors (n=4), 7.1±2.4 (3.5-10.1) in mediastinal carcinoid (n=11), and 2.0±0.1 (1.9-2.2) in bronchial carcinoid (n=4). This study first reported the F-FDG PET/CT images of ACTH-secreting olfactory neuroblastoma. Serum ACTH level determines the success rate of localization of the primary ACTH-secreting tumor in ACTH-dependent syndrome. F-FDG PET/CT plays a role in localizing the site for EAS, although it plays a limited role in Cushing disease.

Cushing disease in children and adolescents - assessment of the clinical course, diagnostic process, and effects of the treatment - experience from a single paediatric centre.

Adrenocorticotropic hormone-dependent Cushing syndrome, known as Cushing disease (CD), is a rare disease in paediatric patients, the signs and symptoms of which differ from those seen in adult patients. The objective of the study was to present the diagnostics and treatment results of CD in children and adolescents. We included four consequent patients in the study, 7-15 years old, with CD confirmed by laboratory tests and finally by histology ex-aminations after surgery. The data were retrospectively retrieved from the medical records of all the patients from the years 2012-2018. The median time from the onset of clinical symptoms to CD diagnosis was 1.5 years. All patients presented with rapid weight gain ac-companying growth retardation, and behavioural, puberty, or menstruation disorders. The diagnostic process was carried out in accord-ance with the current standard. Diagnostic difficulties regarded the cyclic CD. All patients underwent transsphenoidal complete resection of the pituitary adenoma, and they all required transient glucocorticoid substitution for a period that correlated with the time from onset of symptoms to diagnosis. All subjects achieved normalisation of hormonal results and resolution of clinical symptoms, but all are still obese. Currently the patients are in remission, but there is a suspicion of the regrowth of the tumour mass in the eldest patient. In each case of the coexistence of growth velocity inhibition (not necessarily short stature) with the weight gain (not necessarily obesity) and the appearance of hyperandrogenism and depressive-anxiety disorders, biochemical diagnostics of CS should be performed. Obesi-ty could be a chronic complication of CD in childhood, even after effective neurosurgery treatment.

RELIABILITY OF THE CORTICOTROPIN RELEASING HORMONE STIMULATION TEST FOR DIFFERENTIATING BETWEEN ACTH DEPENDENT AND INDEPENDENT CUSHING SYNDROME.

It is a challenge to determine the origin of Cushing syndrome (CS), especially in patients with low-normal adrenocorticotropic hormone (ACTH) concentrations. To evaluate the reliability of the corticotropin-releasing hormone (CRH) stimulation test in patients with CS whose origin of disease was not clearly identified using ACTH values, the high-dose dexamethasone suppression test (HDDST), and imaging in a single tertiary referral center. Twenty-one patients with CS who were admitted to the endocrinology-metabolism clinic between 2004 and 2016 whose ACTH concentrations were 5-20 pg/mL and needed CRH stimulation test were retrospectively assessed. Nine out of 21 patients were diagnosed as having Cushing's disease (CD) and 12/21 had adrenal CS. The CRH stimulation test had a sensitivity and specificity of 100% and 8%, and positive and negative predictive values of 100% and 45% according to the current diagnostic criteria, respectively. An increase in ACTH ≥115% at 15 minutes and cortisol ≥86% at 60 minutes after CRH were associated with the highest likelihood ratio. The sensitivity and specificity of ACTH was 67% and 83% (AUC=0.75±0.12, 95% CI: [0.5-0.9]; p=0.03), and for cortisol it was 75% and 78% (AUC=0.71±0.15, 95% CI: [0.5-0.9]; p=0.03). Cortisol suppression of more than 64% from basal level in the HDDST suggested CD with the highest likelihood ratio. When these cut-off values were used together, both tests were negative in the patients with CD. The CRH stimulation test has low specificity to localize CS in patients with ACTH concentrations of 5-20 pg/mL according to the current diagnostic criteria. Different diagnostic criteria may be used in the CRH stimulation test and also in the HDDST in this group of patients.

The localization diagnosis of patients with adrenocorticotropic hormone-dependent Cushing's syndrome in adolescence

Objective: To elucidate the clinical characteristics and localization diagnosis of patients with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) in adolescence. Methods: The clinical data, laboratory examination and localization diagnosis from 35 patients aged less than 18 years old with adolescent CS who were treated at Peking Union Medical College Hospital between January 1990 and March 2012 were analyzed. Results: There were 29 cases of Cushing's disease (CD) and 6 cases of ectopic ACTH syndrome (EAS). Compared to patients with EAS, those with CD were older at diagnosis[(15.2±2.7) vs (12.8±4.4) years], and had longer disease course[(1.9±1.5) vs (0.7±0.3) years]and higher serum potassium[(3.8±0.6) vs (2.5±0.7) mmol/L], however the plasma ACTH level[(15.4±14.9) vs (42.5±22.7) pmol/L]was lower (all P<0.05). If the cut-off of the ratio of 24-hour urine free cortisol (24 h UFC) after low-dose dexamethasone suppression test (LDDST) to before LDDST was 0.65, the sensibility to diagnose CD was 70.8%, and the specificity was 100%. If the cut-off of the 24 h UFC ratio after high-dose dexamethasone suppression test (HDDST) to before HDDST was 0.54, the sensibility to diagnose CD was 91.7%, and the specificity was 100%. If the cut-off of the plasma ACTH ratio of inferior petrosal vein[bilateral inferior petrosal sinus sampling (BIPSS)]to peripheral vein was 2, only 6 CD patients (6/8) met it. Conclusion: The study suggested that HDDST was more meaningful in the localization diagnosis of patients with ACTH-dependent CS in adolescence.

Carbon dioxide combining power/potassium ratio in etiological diagnosis of Cushing's syndrome

Objective To evaluate carbon dioxide combining power/potassium ratio (CO2CP/K)in etiological diagnosis of Cushing's syndrome. Methods The clinical data 489 patients with pathohistologically confirmed Cushing's syndrome treated in PLA General Hospital between 1993 and 2015 were retrospectively reviewed. Among 489 patients, there were 246 cases of adrenocorticotropic hormone(ACTH) nondependent Cushing's syndrome and 243 cases of ACTH dependent Cushing's syndrome, including 214 cases of Cushing's disease (CD) and 29 cases of ectopic ACTH syndrome (EAS). Receiver operating characteristic(ROC) curve was used to evaluate efficacy of CO2CP/K for etiological diagnosis and the cut-off value and corresponding sensitivity and specificity were analyzed. Results Blood potassium levels were lower in ACTH nondependent group than those in ACTH dependent group[(3.46±0.77) vs.(3.69±0.57)mmol/L, t=3.433, P=0.001]. Bicarbonate, base excess and CO2CP/K (10.0±5.5 vs. 7.6±2.1) were higher in ACTH nondependent group than those in ACTH dependent group (t=-3.612, P=0.000). There was no significant difference in serum sodium, carbon dioxide binding force and pH value between two groups (P>0.05). Blood potassium [(2.47±0.63) vs.(3.62±0.67)mmol/L] and chlorine levels were lower in EAS group than those in CD group. Bicarbonate, base excess and CO2CP/K (15.6±8.0 vs. 7.8±2.4) were higher in EAS group than those in CD group (t=- 10.145, P=0.000). There was no significant difference in serum sodium, carbon dioxide binding force and PH value between two groups (P>0.05). Taking 8.95 as cut-off value the sensitivity and specificity of CO2CP/K in diagnosis of ACTH dependent Cushing's syndrome were 0.34 and 0.85, while taking 9.09 as cut-off value the sensitivity and specificity of CO2CP/K in diagnosis of EAS were 0.89 and 0.78, respectively. Conclusions The value of CO2CP/K is limited in distinguishing between ACTH dependent and non-ACTH independent Cushing, s syndrome. It may distinguish CD from EAS to some extent, indicating that it might be used as the screening method for etiological diagnosis. Key words: Cushing's syndrome; Hypokalemia; Etiology; Diagnosis; Adrenal gland neoplasms

Etiology analysis of 522 hospitalized cases with Cushing syndrome

To summarize the characteristics of the etiology distribution of Cushing syndrome (CS) patients in Chinese PLA general hospital during the past 20 years. All data of CS patients admitted to Chinese PLA General Hospital between 1995 and 2014 were collected. The sex ratio of patients, visiting age, final diagnosis were retrospectively analyzed. From January 1995 to December 2014, 522 patients with CS were admitted, of which, 134 (25.67%) were males and 388(74.33%) were females. The overall mean age was (42±13) years old. 46.36% of the cases were adrenocorticotropic hormone (ACTH) dependent, and ACTH independent CS accounted for 53.64%. At the 41-60 age group, the proportion of ACTH independent CS was higher than that of ACTH independent CS.The proportions of ACTH-independent macronodular adrenal hyperplasia (AIMAH), ectopic ACTH syndrome and adrenal cortical carcinoma were higher in male group than in female group. The number of CS patients was gradually increased during the past 20 years. The proportion of ACTH independent CS was increased (from 32.8% to 60.4%). The proportion of ACTH dependent CS was declined (from 67.2% to 39.6%). The etiology spectrum of CS has changed. There was significant gender-related, age-related difference of etiology distribution in patients with CS.

Single-port robotic-assisted adrenalectomy: feasibility, safety, and cost-effectiveness.

Background and Objectives:The introduction of robotic surgery offers patients and surgeons new options for adrenalectomy. Whereas multiport adrenalectomies have been safely performed using the robot, we describe our experience with the novel technique of single-port robotic-assisted adrenalectomy.Methods:We performed a matched-cohort study comparing 16 single-port robotic-assisted adrenalectomies with 16 patients from a pool of 148 laparoscopic adrenalectomies, matched for age, gender, operative side, pathology, and body mass index. All were operated on by 1 surgeon.Results:The pathology included aldosteronoma in 44% of patients, adrenocorticotropic hormone–dependent Cushing syndrome (bilateral adrenalectomy) in 19%, pheochromocytoma in 13%, and other pathology in 24%. The operative time was 183 ± 33 minutes for single-port robotic-assisted adrenalectomy and 173 ± 40 minutes for laparoscopic adrenalectomy (P = .58). The total time in the operating room was 246 ± 33 minutes for single-port robotic-assisted adrenalectomy and 240 ± 39 minutes for laparoscopic adrenalectomy (P = .57). There was 1 conversion to open adrenalectomy (6%) in each group, both because of bleeding on the right side during bilateral adrenalectomy. Two right-sided single-port robotic-assisted adrenalectomy patients required conversion to laparoscopic adrenalectomy, one because of poor visualization. There were no deaths. Complications occurred in 2 patients in each group (intensive care unit admission, prolonged ileus). Both groups had similar pain scores (mean of 3.7 on a scale from 1 to 10) on postoperative day 1, and patients in the single-port robotic-assisted adrenalectomy group used less narcotic pain medication in the first 24 hours after surgery (43 mg vs 84 mg in laparoscopic adrenalectomy group, P < .001). The differences between the single-port robotic-assisted adrenalectomy group and laparoscopic adrenalectomy group in length of stay (2.3 ± 0.5 days vs 3.1 ± 0.9 days, P = .23), percentage of patients discharged on postoperative day 1 (56% vs 31%, P = .10), and hospital cost (16% lower in single-port robotic-assisted adrenalectomy group, P = .17) did not reach statistical significance.Conclusion:Single-port robotic adrenalectomy is feasible; patients require less narcotic pain medication whereas costs appear equivalent compared with laparoscopic adrenalectomy.

A Case of Ectopic Adrenocorticotropic Hormone Syndrome in Small Cell Lung Cancer.

Small cell lung cancer (SCLC), which originated from neuroendocrine tissue, can develop into paraneoplastic endocrine syndromes, such as Cushing syndrome, because of an inappropriate secretion of ectopic adrenocorticotropic hormone (ACTH). This paraneoplastic syndrome is known to be a poor prognostic factor in SCLC. The reason for poor survival may be because of a higher risk of infection associated with hypercortisolemia. Therefore, early detection and appropriate treatment for this syndrome is necessary. But the diagnosis is challenging and the source of ACTH production can be difficult to identify. We report a 69-year-old male patient who had severe hypokalemia, metabolic alkalosis, and hypertension as manifestations of an ACTH-secreting small cell carcinoma of the lung. He was treated with ketoconazole and spironolactone to control the ACTH dependent Cushing syndrome. He survived for 15 months after chemotherapy, which is unusual considering the poor outcome of the ectopic ATH syndrome associated with SCLC.