Factors predicting the duration of adrenal insufficiency in patients successfully treated for Cushing disease and nonmalignant primary adrenal Cushing syndrome

Endocrine ◽  
2016 ◽  
Vol 55 (3) ◽  
pp. 969-980 ◽  
Author(s):  
Alessandro Prete ◽  
Rosa Maria Paragliola ◽  
Filomena Bottiglieri ◽  
Carlo Antonio Rota ◽  
Alfredo Pontecorvi ◽  
...  
Keyword(s):  
Adrenal Insufficiency ◽  
Cushing Syndrome ◽  
Cushing Disease

scholarly journals Mifepristone as Bridge or Adjunct Therapy in the Management of Challenging Cushing Disease Cases

2021 ◽  
Vol 14 ◽  
pp. 117955142199410
Author(s):  
Alice Y Chang ◽  
Sasan Mirfakhraee ◽  
Elizabeth E King ◽  
Jennifer U Mercado ◽  
Diane M Donegan ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Severe Disease ◽  
Disease Recurrence ◽  
Adjunctive Treatment ◽  
Therapeutic Trial ◽  
Cushing Disease ◽  
The Us ◽  
Clinical Benefits ◽  
Effects Of Radiation ◽  
Bridge Therapy

Establishing a definitive diagnosis of Cushing disease (CD), given its clinical and biochemical heterogeneity, initiating effective treatment to control the effects of hypercortisolism, and managing recurrence are challenging disease aspects to address. Mifepristone is a competitive glucocorticoid receptor antagonist that is approved in the US by the Food and Drug Administration to control hyperglycemia secondary to endogenous hypercortisolism (Cushing syndrome) in patients who have glucose intolerance or type 2 diabetes mellitus and have failed surgery or are not candidates for surgery. Herein, we describe 6 patients with CD who received mifepristone as adjunct/bridge therapy in the following clinical settings: to assess clinical benefits of treatment for suspected recurrent disease, to control hypercortisolism preoperatively for severe disease, to control hypercortisolism during the COVID-19 pandemic, and to provide adjunctive treatment to radiation therapy. The patients were treated at multiple medical practice settings. Mifepristone treatment in each of the described cases was associated with clinical improvements, including improvements in overall glycemia, hypertension, and weight loss. In addition, in one case where biochemical and radiological evidence of disease recurrence was uncertain, clinical improvement with mifepristone pointed toward likely disease recurrence. Adverse events associated with mifepristone reported in the 6 cases were consistent with those previously reported in the pivotal trial and included cortisol withdrawal symptoms, antiprogesterone effects (vaginal bleeding), hypothyroidism (treated with levothyroxine), and hypokalemia (treated with spironolactone). These cases show how mifepristone can potentially be utilized as a therapeutic trial in equivocal cases of CD recurrence; as a presurgical treatment strategy, particularly during the COVID-19 pandemic; and as bridge therapy, while awaiting the effects of radiation.

Adrenal Hypertension

2017 ◽  
Author(s):  
Naomi D.L. Fisher ◽  
Gail K Adler
Keyword(s):  
Myocardial Infarction ◽  
Adrenal Glands ◽  
Cushing Syndrome ◽  
Rare Tumor ◽  
Cushing Disease ◽  
Increased Risk ◽  
Aldosterone Production ◽  
Artery Disease ◽  
Secondary Causes ◽  
Adrenal Hypertension

The secondary causes of hypertension are associated with the excess of the principal hormones produced by the adrenal glands: cortisol, epinephrine, and aldosterone. Excess aldosterone production is recognized as primary hyperaldosteronism, or primary aldosteronism (PA). Individuals with PA are at increased risk for a variety of disorders, including atrial fibrillation, coronary artery disease, myocardial infarction, and stroke. Pheochromocytoma is a very rare tumor (accounting for fewer than one in 10,000 hypertension cases) and is marked by high secretions of catecholamines, mostly epinephrine as well as norepinephrine. Cushing disease and Cushing syndrome are addressed in a separate review. This review contains 5 highly rendered figures, 4 tables, and 39 references.

Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases

2004 ◽  
Vol 100 (4) ◽  
pp. 634-638 ◽  
Author(s):  
Charlotte Höybye ◽  
Eva GrenbäcK ◽  
Marja Thorén ◽  
Anna-Lena Hulting ◽  
Lars Lundblad ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Transsphenoidal Surgery ◽  
Cushing Syndrome ◽  
Preoperative Evaluation ◽  
Clinical Signs ◽  
Pituitary Hormone ◽  
Years Of Experience ◽  
Cushing Disease ◽  
Content Type ◽  
Fine Print

Object. Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)—producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon. Methods. Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13–74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 ± 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention. Conclusions. Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.

scholarly journals Otolaryngic Manifestations of Cushing Disease

2017 ◽  
Vol 96 (8) ◽  
pp. E28-E30 ◽  
Author(s):  
Edward C. Kuan ◽  
Kevin A. Peng ◽  
Jeffrey D. Suh ◽  
Marvin Bergsneider ◽  
Marilene B. Wang
Keyword(s):  
Pituitary Adenoma ◽  
Cushing Syndrome ◽  
Medical Center ◽  
Academic Medical Center ◽  
Retrospective Chart Review ◽  
Signs And Symptoms ◽  
Cushing Disease ◽  
Comprehensive Management ◽  
Academic Medical ◽  
Obstructive Sleep

Cushing disease is a relatively rare cause of Cushing syndrome secondary to a hyperfunctioning pituitary adenoma. In addition to signs and symptoms of hypercortisolism, Cushing disease may present with diverse otolaryngic manifestations, which may guide diagnosis and management. We performed a retrospective chart review of patients who were found to have Cushing disease and who underwent transnasal transsphenoidal surgery for pituitary adenomas between January 1, 2007, and July 1, 2014, at a tertiary academic medical center. There were 37 consecutive patients in this series with Cushing disease caused by a pituitary adenoma. Fifteen (41%) patients complained of visual changes. Five (14%) patients suffered from obstructive sleep apnea. Four (11%) patients had thyroid disease. Other symptoms included hearing loss, vertigo, tinnitus, epistaxis, dysphagia, and salivary gland swelling. Although Cushing disease traditionally presents with classic “Cushingoid” systemic features, it also may present with various otolaryngic manifestations. A thorough workup by otolaryngologists is critical in the comprehensive management of these patients.

scholarly journals Adrenocorticotropic Hormone-Dependent Cushing Syndrome Caused by an Olfactory Neuroblastoma

2019 ◽  
Vol 12 ◽  
pp. 117955141982583 ◽  
Author(s):  
Cristina Familiar ◽  
Ane Azcutia
Keyword(s):  
Adrenocorticotropic Hormone ◽  
Cushing Syndrome ◽  
Combined Therapy ◽  
Malignant Neoplasm ◽  
Olfactory Neuroblastoma ◽  
Large Mass ◽  
High Dose ◽  
Cushing Disease ◽  
Fractionated Radiotherapy ◽  
High Dose Dexamethasone

Olfactory neuroblastoma (ONB) is an unusual malignant neoplasm originating from the olfactory neuroepithelium. Secretion of adrenocorticotropic hormone (ACTH) from this tumor has been exceptionally reported. We describe a young man with resistant hypertension and a cushingoid phenotype. After hormonal confirmation of an ACTH-dependent Cushing syndrome, non-invasive dynamic tests were carried out to evaluate the cause of the ACTH source. Plasma cortisol decrease after a high-dose dexamethasone suppression test and cortisol increase after a desmopressin (DDAVP) stimulation test suggested a Cushing disease. A magnetic resonance image (MRI) of the brain and an Indium-111 octreotide scan revealed a large mass centered in the sphenoid sinus with lateral and posterior extension. An ACTH secreting ONB was confirmed with a trasnasal biopsy. Patient was offered a combined therapy with surgical resection and radiotherapy but refused surgery. The neoplasm was treated with neoadjuvant cisplatin-based chemotherapy followed by fractionated radiotherapy. Hypercortisolism initially improved with metyrapone but normocortisolism was only achieved after local control of the tumor with radiotherapy. Clinical presentation of ONB is usually related to local symptoms (as nasal obstruction and epistaxis) dependent on its ubication and extension. Cushing syndrome from ACTH production is a rare manifestation of ONB. This case also underlies the difficulties related to the interpretation of dynamic endocrine tests in Cushing syndrome.

scholarly journals Presentation, Treatment, Histology, and Outcomes in Adrenal Medullary Hyperplasia Compared With Pheochromocytoma

2019 ◽  
Vol 3 (8) ◽  
pp. 1518-1530 ◽  
Author(s):  
Henrik Falhammar ◽  
Adam Stenman ◽  
Jan Calissendorff ◽  
Carl Christofer Juhlin
Keyword(s):  
Blood Pressure ◽  
Surgical Management ◽  
Cushing Syndrome ◽  
Adrenocortical Adenoma ◽  
Cushing Disease ◽  
Genetic Syndrome ◽  
Alpha Blockers ◽  
Adrenal Medullary Hyperplasia ◽  
Symptoms And Signs

Abstract Context Information about adrenal medullary hyperplasia (AMH) is scarce. Objective To study a large cohort of AMHs. Design, Setting, and Participants Nineteen AMH cases were compared with 95 pheochromocytomas (PCCs) without AMH. AMH without (n = 7) and with PCC (n = 12) were analyzed separately. Results Of 936 adrenalectomies, 2.1% had AMH. Mean age was 47.2 ± 15.1 years. Only two (11%) AMHs had no concurrent PCC or adrenocortical adenoma. In AMHs, a genetic syndrome was present in 58% vs 4% in PCCs (P < 0.001). The noradrenaline/metanephrine levels were lower in AMHs, whereas suppression of dexamethasone was less than in PCCs. Cushing syndrome was found in 11% of AMHs. More AMHs were found during screening and less as incidentalomas. PCC symptoms were less prevalent in AMHs. Surgical management was similar; however, fewer of the AMHs were pretreated with alpha-blockers. Adrenalectomy improved blood pressure slightly less in AMHs. The disappearance of glycemic disturbances was similar to the PPCs. During a period of 11.2 ± 9.4 years, a new PCC developed in 32% of patients with AMH, 11% died, but no PCC metastasis occurred (PCCs: 4%, P < 0.001; 14% and 5%). AMHs without PCC had milder symptoms but more often Cushing disease than patients with PCC, whereas AMH with PCC more often displayed a familiar syndrome with more PCC recurrences. Conclusion A total of 2.1% of all adrenalectomies displayed AMH. AMH seemed to be a PCC precursor. The symptoms and signs were milder than PCCs. AMHs were mainly found due to screening. Outcomes seemed favorable, but new PCCs developed in many during follow-up.

To determine the efficacy of bilateral inferior petrosal sinus sampling in differentiating Cushing disease from ectopic Cushing syndrome

2020 ◽  
pp. 1-11
Author(s):  
Muhammad Azeemuddin ◽  
Tanveer Ul Haq ◽  
Shahmeer Khan ◽  
Raza Sayani ◽  
Ayesha Shoukat Hussain ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Case Series ◽  
Invasive Procedure ◽  
University Hospital ◽  
Inferior Petrosal Sinus ◽  
Cushing Disease ◽  
Post Contrast ◽  
Inferior Petrosal Sinus Sampling ◽  
Positive Results

Abstract Management of endogenous Cushing syndrome is based on its aetiology. Increased Adrenocorticotropic Hormone (ACTH) levels are the most common cause of this disorder and, therefore, it is critical to determine the source of ACTH before further management. Dynamic post contrast MRI is currently the most common investigation implied to diagnose pituitary adenoma; however, it comes with the drawback of low specificity and high false positive results. Inferior petrosal sinus sampling (IPSS) is an established invasive procedure performed to differentiate central versus peripheral source of ACTH which, in turn, results in hypercortesolaemia. This is a series of 14 patients who underwent IPSS at the Department of Radiology, Aga Khan University Hospital, Karachi, from January 2006 to December 2018. The case series emphasises the role of IPSS in the management of ACTH-dependent Cushing syndrome and combined efficacy of Dynamic post-contrast MRI and the procedure under focus. Continuous....

scholarly journals MON-188 The Diagnostic Value of DHEAS in Subtyping Patients with Cushing Syndrome

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Mengsi Liu ◽  
keying Zhu ◽  
Huan Chen ◽  
Wenhuan Feng ◽  
Dalong Zhu ◽  
...  
Keyword(s):  
Cushing Syndrome ◽  
Laboratory Data ◽  
Area Under The Curve ◽  
Dehydroepiandrosterone Sulfate ◽  
Diagnostic Value ◽  
Acth Level ◽  
Plasma Acth ◽  
Clinical Value ◽  
Cushing Disease ◽  
Diagnostic Sensitivity And Specificity

Abstract Objective: Serum dehydroepiandrosterone sulfate (DHEAS) can be used to assess the integrity of the hypothalamic-pituitary-adrenal (HPA) axis. The aim of this study was to evaluate the clinical value of DHEAS in differentiating adrenal Cushing syndrome (ACS) from Cushing’ disease (CD). Methods: We recruited 100 patients with Cushing syndrome, 36 with CD and 64 with ACS. 72 sex-, age- and BMI-matched nonfunctional adrenal adenomas (NFAAs) were served as controls. Clinical and laboratory data were collected. DHEAS levels were measured and DHEAS ratio was calculated by dividing the measured DHEAS by the lower limit of the respective reference range (age- and sex-matched). Results: 1) No significant differences in age, sex, or BMI were detected among the NFAAs, ACS and CD groups. Compared to NFAAs group, ACS patients had lower plasma ACTH levels [1.11(1.11,1.74) vs 5.0 ± 2.9 pmol/L, P&lt;0.01], lower DHEAS levels (24.00 ± 20.72 vs 189.05 ± 82.03 ug/dL, P &lt; 0.01) and lower DHEAS ratio [0.58(0.27,0.98) vs 5.34 ± 3.0]; Plasma ACTH (22.12 ± 14.22 pmol/L), DHEAS (309.4 ± 201.1 ug/dL) and DHEAS ratio (10.51 ± 7.65) in CD patients were significantly higher compared to those in NFAAs and ACS patients (all P&lt;0.01). 2) In ACS patients, there were 53 patients with suppressed ACTH level of &lt;2.0 pmol/L, 11 patients without plasma ACTH suppression (≥2.0pmol/L). Compared to NFAAs, lower DHEAS and DHEAS ratio were detected in these two groups, and no significant differences were found in the DHEAS [15(15, 23.5) vs 23.8 ± 14.4 ug/dL, P=0.86] and DHEAS ratio [0.58(0.27, 0.80) vs 1.0(0.25,2.09) ug/dL, P=0.40] between the two groups. 3) ROC analysis showed that the area under the curve (AUC) of plasma ACTH, serum DHEAS and DHEAS ratio in diagnosing 0.954, 0.997 and 0.990 respectively. The optimal cut-off values for DHEAS and its ratio were 79.1ug/dL, and 2.09, respectively. The diagnostic sensitivity and specificity of plasma ACTH (&lt;2.0pmol/L) were 84.1 and 100%, those of DHEAS were 97.5% and 100%, and those of DHEAS ratio were 95% and 100%, respectively. Conclusions: Patients with different subtype of Cushing syndrome showed distinctive DHEAS levels and DHEAS ratio. DHEAS and DHEAS ratio are useful in differential diagnosis of Cushing syndrome. Especially, when the plasma ACTH level is not conclusive. The measurement of DHEAS may offer a supplementary test to diagnosis ACS from CD. Keywords: Adrenal Cushing syndrome; Cushing disease; Adrenocorticotropic hormone; Dehydroepiandrosterone sulfate

scholarly journals The Incidence of Long-Term Adrenal Insufficiency Following Unilateral Adrenalectomy is Low: Case Series and Review of Literature

2019 ◽  
pp. 1-4
Author(s):  
Jay D. Raman ◽  
Augustyna Gogoj ◽  
Brian D. Saunders ◽  
Daniel J.Canter ◽  
Jay D. Raman ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Adrenal Insufficiency ◽  
Cushing Syndrome ◽  
Case Series ◽  
Large Majority ◽  
Review Of Literature ◽  
Unilateral Adrenalectomy ◽  
Different Types ◽  
One Year

Introduction: Acquired adrenal insufficiency is a known risk of unilateral adrenalectomy. However, the rates of early and prolonged adrenal insufficiency following unilateral adrenalectomy are not well defined in the literature. Patients and Methods: We reviewed a case series of 184 consecutive patients to determine the likelihood of steroid supplementation at 30 days and 1 year following adrenalectomy. 109 lesions were non-functional and 75 (41%) demonstrated functionality, including 33 pheochromocytomas, 20 cortisol-producing adenomas, 19 aldosteronomas, and 3 cases of cortisol-secreting hyperplasia. No patients with a nonfunctional lesion, pheochromocytoma, or aldosteronoma required steroid supplementation following surgery. Eleven of 23 patients (48%) with primary adrenal Cushing syndrome required cortisol supplementation at 30 days, and only 1 patient (4%) necessitated supplementation one year following surgery. Discussion: Approximately 50% of patients with cortisol-producing lesions in the adrenal gland will require supplementation 30-days following surgery. Only 4% will require persistent exogenous steroids at 1-year. Conversely, less than 1% of patients with different types of functional or non-functional tumors required supplementation after surgery. Conclusion: The incidence of adrenal insufficiency following unilateral adrenalectomy is low. A large majority of patients requiring steroid supplementation 30 days following surgery are able to wean off this requirement by 1 year. With this information, we can better counsel our patients and set clearer expectations for the potential need of cortisol supplementation following adrenalectomy

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