Diagnosis Of Cushing's Syndrome Research Articles

Conditioning Factors for High Cardiovascular Risk in Patients with Cushing Syndrome

To characterize the alterations in carbohydrate and lipoprotein metabolism, to evaluate markers of lipoprotein functionality, and to identify the presence of novel atherogenic risk factors in patients with Cushing syndrome (CS) in comparison with sex- and age-matched controls. In an open, cross-sectional study, 32 nontreated patients with active CS were consecutively recruited from the Endocrinology Service at "José de San Martín" Clinical Hospital, University of Buenos Aires, Argentina, between April 11, 2010 and December 11, 2012. The patients were compared with sex- and age-matched controls. Versus controls, patients with CS presented with excess weight, central obesity, and hypercortisolism. They also exhibited an insulin-resistant state, with high resistin levels (median [interquartile range], 16 [10 to 22] ng/mL versus 6 [5 to 9] ng/mL; P<.0001), a more atherogenic lipoprotein profile, high oxidized low-density lipoprotein levels (oxLDL; mean ± SD, 100 ± 31 U/L versus 75 ± 32 U/L; P<.05) and high sensitive C-reactive protein levels (median [interquartile range], 1.2 [0.6 to 3.1] mg/L versus 0.6 [0.3 to 1.1] mg/L; P<.05), and increased leukocyte count (mean ± SD, 9.5 ± 2.6 × 10(3) cells/μL versus 6.5 ± 1.4 × 10(3) cells/μL; P<.0001). Multivariate analyses showed that the increase in waist circumference was associated with both the diagnosis of CS and the degree of insulin resistance. Resistin concentration was related to a greater extent to the diagnosis of CS than to homeostasis model assessment-insulin resistance. Triglyceride and oxLDL levels were only significantly associated with the diagnosis of CS. Hypercortisolism is related to the increase observed in triglycerides and oxLDL levels, and, in combination with insulin resistance, acts to increase waist circumference and amplify the inflammatory process, key factors for the development of cardiovascular disease.

MANAGEMENT OF ENDOCRINE DISEASE: Management of pregnant patients with Cushing's syndrome.

Progress in the diagnosis and treatment of endocrine diseases has turned pregnancy into a possibility for women with such medical disorders, including Cushing's syndrome (CS). Nevertheless, despite its rarity, pregnancy in patients with CS can be troublesome because of the risk of maternal-fetal complications. Therefore, hypercortisolism, if present, should be surgically or medically controlled in most cases. Moreover, changes in the hypothalamic-pituitary-adrenal axis during normal pregnancy may mislead the diagnosis of CS during this period, because many laboratory assessments suggestive of CS may be present in normal pregnancy, with clinical features mimicking those seen in patients with CS. The aim of the present review is to update the diagnostic approach to this medical condition, mainly for pregnant women without previous diagnosis of CS, and to describe the therapeutic strategies for CS during pregnancy in order to minimize complications for both mother and fetus.

Treatment of canine and feline hyperadrenocorticism: trilostane and the alternatives

The common causes of hyperadrenocorticism (HAC) are pituitary ACTH-secreting corticotroph tumours, known as pituitary-dependent hyperadrenocorticism, and cortisol-secreting adrenal tumours. The only licensed medical treatment in the UK is trilostane. This treatment improves the clinical signs of HAC in the majority of cats and dogs. There are a number of alternative treatment options that are available for use in non-responders or as first-line treatment instead of trilostane. After a diagnosis of HAC is made, each option should be discussed with clients. This article discusses medical, surgical and radiotherapy options that should be considered to create an individualised treatment plan for each patient and owner.

Evolution, Global Warming, Smart Phones, and Late-Night Salivary Cortisol

Recently, I was asked to see a young woman for a second opinion regarding the possible diagnosis of Cushing syndrome. She had already been evaluated by another endocrinologist who had dismissed the diagnosis because the patient’s urine free cortisol was normal. When I asked the patient whether a measurement of late-night salivary cortisol (LNSC) had been secured, she replied, “The endocrinologist I saw doesn’t believe in salivary cortisol.” Having heard this on other occasions, I smiled and replied, “There are people who do not believe in evolution or global warming, but the scientific evidence for these phenomena is very persuasive.” Similarly, the scientific evidence now for the diagnostic utility of LNSC in the evaluation of patients with suspected endogenous hyper-cortisolism is very strong.

Limited Diagnostic Utility of Plasma Adrenocorticotropic Hormone for Differentiation between Adrenal Cushing Syndrome and Cushing Disease.

BackgroundMeasurement of the plasma adrenocorticotropic hormone (ACTH) level has been recommended as the first diagnostic test for differentiating between ACTH-independent Cushing syndrome (CS) and ACTH-dependent CS. When plasma ACTH values are inconclusive, a differential diagnosis of CS can be made based upon measurement of the serum dehydroepiandrosterone sulfate (DHEA-S) level and results of the high-dose dexamethasone suppression test (HDST). The aim of this study was to assess the utility of plasma ACTH to differentiate adrenal CS from Cushing' disease (CD) and compare it with that of the HDST results and serum DHEA-S level.MethodsWe performed a retrospective, multicenter study from January 2000 to May 2012 involving 92 patients with endogenous CS. The levels of plasma ACTH, serum cortisol, 24-hour urine free cortisol (UFC) after the HDST, and serum DHEA-S were measured.ResultsFifty-seven patients had adrenal CS and 35 patients had CD. The area under the curve of plasma ACTH, serum DHEA-S, percentage suppression of serum cortisol, and UFC after HDST were 0.954, 0.841, 0.950, and 0.997, respectively (all P<0.001). The cut-off values for plasma ACTH, percentage suppression of serum cortisol, and UFC after HDST were 5.3 pmol/L, 33.3%, and 61.6%, respectively. The sensitivity and specificity of plasma ACTH measurement were 84.2% and 94.3%, those of serum cortisol were 95.8% and 90.6%, and those of UFC after the HDST were 97.9% and 96.7%, respectively.ConclusionSignificant overlap in plasma ACTH levels was seen between patients with adrenal CS and those with CD. The HDST may be useful in differentiating between these forms of the disease, especially when the plasma ACTH level alone is not conclusive.

Death in pediatric Cushing syndrome is uncommon but still occurs.

Cushing syndrome (CS) in children is rare. Delayed diagnosis and treatment of CS may be associated with increased morbidity and, unfortunately, mortality. We performed a retrospective review of all patients with CS under the age of 18years referred to the National Institutes of Health (NIH) from 1998 to 2013 in order to describe deceased patients among cases of pediatric CS referred to the National Institutes of Health (NIH). The deaths of four children (three females and one male), aged 7.5-15.5years (mean age 11.2years) with length of disease 2-4years, were recorded among 160 (2.5%) children seen at or referred to the NIH over the last 15years. All died at different institutions, prior to coming to the NIH (two) or after leaving NIH (two). Presenting symptoms included increasing weight and decreasing height gain, facial plethora, dorsocervical fat pad (webbed neck), striae, headache, vision disturbances, and depression and other mood or behavior changes; there were no differences between how these patients presented and the others in our cohort. The causes of CS in the deceased patients were also not different, in fact, they spanned the entire spectrum of CS: pituitary disease (one), ectopic corticotropin production (one), and primary adrenal hyperplasia (one). In one patient, the cause of CS could not be verified. Three died of sepsis and one due to residual disease and complications of the primary tumor. Despite the advances in early diagnosis and treatment of pediatric CS, a 2.5% mortality rate was identified in a large cohort of patients with this condition referred to an experienced, tertiary care referral center (although these deaths occurred elsewhere). Pediatricians need to recognize the possibility of death, primarily due to sepsis, in a patient with pediatric CS and treat accordingly.

Cushing syndrome.

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Incidence of Cushing's syndrome and Cushing's disease in commercially-insured patients

To estimate the incidence of Cushing's syndrome (CS) and Cushing's disease (CD) in the US. MarketScan Commercial database 2007-2010 (age <65 years) was used. CS patients were defined with ≥2 claims of CS diagnosis, while CD patients were defined with CS plus a benign pituitary adenoma diagnosis or hypophysectomy in the same calendar year. Annual incidence was calculated by dividing the number of CS or CD cases by the total number of members with the same enrollment requirement during the calendar years. CS incidence rates per million person-years were 48.6 in 2009 and 39.5 in 2010. The lowest rates of CS were in ≤17-year-olds and highest rates were in 35 to 44-year-olds. CD incidence rates were 7.6 in 2009 and 6.2 in 2010. The lowest rates of CD were in ≤17-year-olds and highest rates were in 18 to 24-year-olds. The rates varied by sex (2.3-2.7 in males, 9.8-12.1 in females). In females, lowest rates ranged 2.5-4.0 in ≤17-year-olds and highest 16.7-27.2 in 18-24 year olds. In males, there were too few cases to report estimates by age. In the first large US-based study, the annual incidence of CS in individuals <65 years old was nearly 49 cases per million, substantially higher than previous estimates, which were based primarily on European data. Using similar methods, we estimated the incidence of CD at nearly 8 cases per million US population. These estimates, if confirmed in other epidemiologic databases, represent a new data reference in these rare conditions.

The diagnostic performance of urinary free cortisol is better than the cortisol:cortisone ratio in detecting de novo Cushing's syndrome: the use of a LC-MS/MS method in routine clinical practice.

The Endocrine Society Clinical Guidelines recommend measuring 24-h urinary free cortisol (UFF) levels using a highly accurate method as one of the first-line screening tests for the diagnosis of Cushing's Syndrome (CS). We evaluated the performance of UFF, urinary free cortisone (UFE), and the UFF:UFE ratio, measured using a liquid chromatography-tandem mass spectrometry (LC-MS/MS) method. The LC-MS/MS was used to analyze UFF and UFE levels in 43 surgically confirmed CS patients: 26 with Cushing's disease (CD, 16 de novo and ten recurrences), 11 with adrenal CS and six with ectopic CS; 22 CD patients in remission; 14 eu-cortisolemic CD patients receiving medical therapy; 60 non-CS patients; and 70 healthy controls. Sensitivity and specificity were determined in the combined groups of non-CS patients, healthy controls, and CD in remission. UFF>170 nmol/24 h showed 98.7% specificity and 100% sensitivity for de novo CS, while sensitivity was 80% for recurrent CD patients, who were characterized by lower UFF levels. The UFF:UFE and UFF+UFE showed lower sensitivity and specificity than UFF. Ectopic CS patients had the highest UFF and UFF:UFE levels, which were normal in the CD remission patients and in those receiving medical therapy. Our data suggest high diagnostic performance of UFF excretion measured using LC-MS/MS, in detecting de novo CS. UFF:UFE and UFF+UFE assessments are not useful in the first step of CS diagnosis, although high levels were found to be indicative of ectopic CS.

Late-night Salivary Cortisol Has a Better Performance Than Urinary Free Cortisol in the Diagnosis of Cushing's Syndrome

The comparison of variability, reproducibility, and diagnostic performance of late-night salivary cortisol (LNSF) and urinary free cortisol (UFC) using concurrent and consecutive samples in Cushing's syndrome (CS) is lacking. Objectives, Patients, and Methods: In a prospective study, we evaluated 3 simultaneous and consecutive samples of LNSF by RIA and UFC by liquid chromatography associated with tandem mass spectrometry in Cushing's disease (CD) patients (n = 43), adrenal CS patients (n = 9), and obese subjects (n = 18) to compare their diagnostic performances. In CS patients, we also performed a modified CS severity index. There was no difference in the coefficient of variation (percentage) between LNSF and UFC among the 3 samples obtained for each patient with Cushing's disease (35 ± 26 vs 31 ± 24), adrenal CS (28 ± 14 vs 22 ± 14), and obesity (39 ± 37 vs 48 ± 20). LNSF confirmed the diagnosis of hypercortisolism even in the presence of normal UFC in 17.3% of CS, whereas the inverse situation was not observed for UFC. The area under the receiver-operating characteristic curves for LNSF was 0.999 (95% credible interval [CI] 0.990-1.00) and for UFC was 0.928 (95% CI 0.809-0.987). The ratio between areas under the curve was 0.928 (95% CI 0.810-0.988), indicating better performance of LNSF than UFC in diagnosing CS. There was no association between the CS severity index and the degree of biochemical hypercortisolism. Our data show that despite similar variability between both methods, LNSF has a superior diagnostic performance than UFC and should be used as the primary biochemical diagnostic test for CS diagnosis.

A Pregnant Woman Who Underwent Laparoscopic Adrenalectomy due to Cushing's Syndrome.

Cushing's syndrome (CS) may lead to severe maternal and fetal morbidities and even mortalities in pregnancy. However, pregnancy complicates the diagnosis and treatment of CS. This study describes a 26-year-old pregnant woman admitted with hypertension-induced headache. Hormonal analyses performed due to her cushingoid phenotype revealed a diagnosis of adrenocorticotropic hormone- (ACTH-) independent CS. MRI showed a 3.5 cm adenoma in her right adrenal gland. After preoperative metyrapone therapy, she underwent a successful unilateral laparoscopic adrenalectomy at 14-week gestation. Although she had a temporary postoperative adrenal insufficiency, hormonal analyses showed that she has been in remission since delivery. Findings in this patient, as well as those in previous patients, indicate that pregnancy is not an absolute contraindication for laparoscopic adrenalectomy. Rather, such surgery should be considered a safe and efficient treatment method for pregnant women with cortisol-secreting adrenal adenomas.

Iatrogenic Cushing Syndrome in Patients Receiving Inhaled Budesonide and Itraconazole or Ritonavir: Two Cases and Literature Review

ObjectiveTo present two cases of iatrogenic Cushing syndrome caused by the interaction of budesonide, an inhaled glucocorticoid, with ritonavir and itraconazole. MethodsWe present the clinical and biochemical data of two patients in whom diagnosis of Cushing syndrome was caused by this interaction. We also reviewed the pertinent literature and management options. ResultsA 71-year-old man was treated with inhaled budesonide for a chronic obstructive pulmonary disease and itraconazole for a pulmonary aspergillosis. The patient rapidly developed a typical Cushing syndrome complicated by bilateral avascular necrosis of the femoral heads. Serum 8:00 AM cortisol concentrations were suppressed at 0.76 and 0.83 µg/dL on two occasions. The patient died 4 days later of a massive myocardial infarction. The second case is a 46-year-old woman who was treated for several years with inhaled budesonide for asthma. She was put on ritonavir, a retroviral protease inhibitor, for the treatment of human immunodeficiency virus (HIV). In the following months, she developed typical signs of Cushing syndrome. Her morning serum cortisol concentration was 1.92 µg/dL. A cosyntropin stimulation test showed values of serum cortisol of <1.10, 2.65, and 5.36 µg/dL at 0, 30, and 60 minutes, respectively, confirming an adrenal insufficiency. Because the patient was unable to stop budesonide, she was advised to reduce the frequency of its administration and eventually taper the dose until cessation. ConclusionClinicians should be aware of the potential occurrence of iatrogenic Cushing syndrome and secondary adrenal insufficiency due to the association of inhaled corticosteroids with itraconazole or ritonavir. (Endocr. Pract. 2013;19:e138-e141)

Disease-specific quality of life evaluation and its determinants in Cushing’s syndrome: what have we learnt?

Cushing’s syndrome (CS) has a considerable negative impact on patient health-related quality of life (HRQoL). Two disease-specific instruments (the CushingQoL and the Tuebingen CD-25 questionnaire) are now available to assess the impact of the disease and its treatment on HRQoL. The purpose of this review was to summarize the characteristics of the studies which have used these two instruments to date and summarize their findings regarding (a) the determinants of disease-specific HRQoL in patients with CS and (b) the impact of treatment for CS on disease-specific HRQoL. A total of 7 studies were identified, 5 with the CushingQoL and 2 with the Tuebingen CD-25. Most were observational studies, though the CushingQoL had been used in one randomized clinical trial. In terms of clinical factors, there was some evidence for an association between UFC levels and disease-specific HRQoL, though the presence and strength of the association varied between studies. There was also some evidence that a more recent diagnosis of CS could lead to poorer HRQoL, and that length of time with adrenal insufficiency may also affect HRQoL. There was no evidence for an impact on disease-specific HRQoL of etiology or of the clinical signs and symptoms associated with CS, such as bruising, rubor, and fat deposits. One factor which did have a significant negative effect on HRQoL was the presence of depression. No clear picture emerged as to the effect of demographic variables such as age and gender on HRQoL scores, though there was some evidence for poorer HRQoL in female patients. As regards treatment, the two interventions studied to date (transsphenoidal surgery and pasireotide) both showed significant gains in HRQoL, with moderate to large effect sizes. This type of review is useful in summarizing knowledge to date and suggesting future research directions.

Detection of salivary cortisol at two different time points at night

Objective To compare the detection results of salivary cortisol(SL) at 23:00 and 24:00 to estimate whether the collection of saliva can be taken in advance at 23:00 for the screening and diagnosis of Cushing's syndrome(CS).Methods Saliva samples were collected by a cotton-based sampling approaches Salivettes at 23:00 and 24:00 from 108 normal subjects (NS group),50 patients (NCS group) with type 2 diabetes,primary hypertension or non-functional adrenal adenoma and 7 patients with Cushing' s syndrome(CS group).Then the samples were assayed by ELISA.Results The levels of SC at 23:00 (SC23) and 24:00 (SC24) in different groups were:2.01 ± 0.91 ng/ml,1.96 ± 0.85 ng/ml in NS group,3.55 ± 1.17 ng/ml,3.81 ± 1.49 ng/ml in NCS group,and 12.63 ± 6.14 ng/ml,10.98 ± 3.98 ng/ml in CS group (all P＞0.05).Using Pearson correlation analysis,SC23 and SC24 showed a very good linear correlation (r =0.924,P＜ 0.001).SC23 or SC24 in CS group were obviously higher than NS and NCS group.The areas under the ROC curves of SC23 and SC24 were 0.995 and 0.996 respectively,which displayed good accuracy to diagnosis of CS.Conclusions Midnight salivary cortisol is a convenient and sensitive indicator for the screening and diagnosis of CS.The concentrations of salivary cortisol at 23:00 and 24:00 are close,so collecting saliva at 23:00 is feasible for the clinical screening and diagnosis of CS. Key words: Salivary cortisol(SC) ; Cushing's Syndrome (CS) ; ELISA

Clinical Characteristics of Endogenous Cushing’s Syndrome at a Medical Center in Southern Taiwan

From January 1987 to December 2011, over a total of 25 years, 84 patients with Cushing's syndrome (CS) were identified at a medical center in southern Taiwan. We observed a higher incidence of ACTH-independent CS (75%) than ACTH-dependent CS (25%). A higher incidence of adrenocortical adenoma (58.3%) than Cushing's disease (CD, 21.4%) was also found. The sensitivity of the definitive diagnostic tests for CS, including loss of plasma cortisol circadian rhythm, a baseline 24 h urinary free cortisol (UFC) value >80 μg, and overnight and 2-day low-dose dexamethasone suppression test, was between 94.4% and 100%. For the 2-day high-dose dexamethasone suppression test for the differential diagnosis of CD, the sensitivity of 0800 h plasma cortisol and 24 h UFC was 44.4% and 85.7%, respectively. For the differential diagnosis of adrenal CS, the sensitivities of the 0800 h plasma cortisol and 24 h UFC were 95.5% and 88.9%, respectively. In patients with ACTH-independent CS and ACTH-dependent CS, the baseline plasma ACTH levels were all below 29 pg/mL and above 37 pg/mL, respectively. The postsurgical hospitalization stay following retroperitoneoscopic adrenalectomy was shorter than that observed for transabdominal adrenalectomy (4.3 ± 1.6 versus 8.8 ± 3.7 days, P < 0.001). It was easy to develop retroperitoneal and peritoneal seeding of adrenocortical carcinoma via laparoscopic adrenalectomy.

Neuroendocrine Carcinoma of the Thyroid Causing Adrenocorticotrophic Hormone–Dependent Cushing's Syndrome

Cushing's syndrome (CS) due to ectopic adrenocorticotrophic hormone (ACTH) and/or ectopic corticotropic releasing hormone (CRH) secretion accounts for <10% of all CS. Neuroendocrine carcinomas rarely cause CS. These carcinomas have been found to secrete either ACTH or rarely CRH. Herein we report a case of neuroendocrine carcinoma originating from the thyroid as the source of ACTH-dependent CS. A 30-year-old woman with features of CS presented with severe respiratory distress. Six months before that, she was diagnosed with primary hypothyroidism and started on levothyroxine (LT4) therapy. Biochemical evaluation was done, and nonsuppressed serum cortisol levels following dexamethasone with high ACTH confirmed a diagnosis of ACTH-dependent CS. Magnetic resonance imaging of the brain showed a bulky pituitary gland. Adrenal imaging showed bilateral adrenal hyperplasia. A computerized tomography scan showed a large anterior mediastinal mass arising from the neck and extending behind the transverse aortic arch. She underwent emergency thoracotomy due to rapidly progressive superior mediastinal syndrome and left vocal cord palsy. At surgery, the mass was seen originating from the thyroid and the thymus was compressed posteriorly. Near total thyroidectomy and thymectomy with removal of pericardial seedlings were done. Histopathology revealed sheets, cords, and nests of round or oval tumor cells with hyperchromatic nuclei and scant cytoplasm with local invasion and lymphovascular embolization suggestive of a neuroendocrine carcinoma arising from thyroid, staining positive for cytokeratin, synaptophysin, and chromogranin-A, and negative for calcitonin and carcinoembryonic antigen. Here we report a case of a neuroendocrine tumor of the thyroid causing ACTH-dependent CS. The tumor was negative for calcitonin staining, indicating that this was not a medullary carcinoma of the thyroid. Neuroendocrine carcinomas originating from the thyroid gland are very rare. A thyroid tumor of neuroendocrine origin causing ACTH-dependent CS has not been reported previously.

Clinical judgment to estimate pretest probability in the diagnosis of Cushing's syndrome under a Bayesian perspective

To estimate the pretest probability of Cushing's syndrome (CS) diagnosis by a Bayesian approach using intuitive clinical judgment. Physicians were requested, in seven endocrinology meetings, to answer three questions: "Based on your personal expertise, after obtaining clinical history and physical examination, without using laboratorial tests, what is your probability of diagnosing Cushing's Syndrome?"; "For how long have you been practicing Endocrinology?"; and "Where do you work?". A Bayesian beta regression, using the WinBugs software was employed. We obtained 294 questionnaires. The mean pretest probability of CS diagnosis was 51.6% (95%CI: 48.7-54.3). The probability was directly related to experience in endocrinology, but not with the place of work. Pretest probability of CS diagnosis was estimated using a Bayesian methodology. Although pretest likelihood can be context-dependent, experience based on years of practice may help the practitioner to diagnosis CS.

The investigation of Cushing syndrome: essentials in optimizing appropriate diagnosis and management.

The investigation of Cushing syndrome (CS) should start with careful history taking and clinical examination, and exogenous steroid usage must be excluded. It is essential to confirm hypercortisolism before further investigations are undertaken. The recommended first-line tests include midnight salivary cortisol and/or the 1 mg overnight or low-dose dexamethasone suppression tests. The next step is to differentiate adrenocorticotrophin (ACTH)-dependent from ACTH-independent CS by measuring ACTH. With ACTH-dependence, further investigations should differentiate pituitary-dependent from ectopic ACTH-dependent CS. Many dynamic tests may be considered, but we suggest that bilateral inferior petrosal sinus sampling (BIPSS) should be performed in almost all patients with ACTH-dependent CS, except for patients with a pituitary macroadenoma. Imaging should include MR scanning of the pituitary, and CT scanning of the chest and abdomen to look for an ectopic source. Confirmation of the diagnosis of CS and accurate localization of its source are vital to optimize therapy for this complex disorder.

A Novel Tool in the Diagnosis and Follow-Up of (Cyclic) Cushing's Syndrome: Measurement of Long-Term Cortisol in Scalp Hair

Measurement of cortisol in 24-h urine collections and midnight saliva are standard screening tests for Cushing's syndrome (CS). These tests reflect cortisol levels during a maximum of 24 h and do not provide historical information. Therefore, they can yield normal results in case of cyclic CS, which is a rare disorder that is characterized by alternating episodes of endogenous cortisol excess and normal cortisol secretion. The measurement of cortisol in scalp hair is a novel tool that might be helpful to establish the diagnosis of (cyclic) CS. Our aim was to study whether hair cortisol timelines correspond with clinical course in patients with CS and whether we could create retrospective timelines of cortisol exposure that correspond with symptomatic periods in patients suspected of cyclic CS. Scalp hair was collected in 14 patients with confirmed CS and six patients suspected of cyclic CS. Cortisol was extracted from the hair samples with methanol, and an ELISA was used to measure cortisol levels in hair extracts. A group of 96 nonobese individuals were used as a control group. Hair cortisol levels were significantly elevated in CS patients (P<0.0001). Sensitivity and specificity of hair cortisol measurements for CS were 86 and 98%, respectively. Hair cortisol timelines of patients with CS and cyclic CS corresponded with clinical course. Hair samples can provide a historical timeline that corresponds with clinical course in patients with (cyclic) CS. This new diagnostic tool can contribute significantly to early recognition of patients suffering from cyclic CS.

A rare case of Cushing syndrome by cyclic ectopic-ACTH

ACTH-dependent Cushing syndrome (CS) due to ectopic ACTH production is most times difficult to manage. The identification of the source of ACTH may take many years. Surgery or chemotherapy for the primary tumor is not always possible. Control of Cushing symptoms is many times achieved using medication, or bilateral adrenalectomy in refractory cases. This case presents a Brazilian male who showed severe hypertension, mood changes, muscle weakness, darkening of skin, and increased abdominal fat. An investigation for Cushing syndrome was carried out and, after a four-year follow-up, a carotid glomus tumor (chemodectoma) was confirmed, a rare ectopic ACTH-producing tumor. Besides, the patient presented cyclic Cushing syndrome that was exacerbated by diverticulitis episodes. This case presents interesting pitfalls on diagnosis and management of ACTH-dependent CS. This is the only report of a chemodectoma that produced ACTH in the literature.