Presentation
A widely used antiacne drug, retinoic acid, can change the clinical presentation of CS and delay the diagnosis. [journals.aace.com]
All patients in whom the ectopic ACTH-secreting tumour was obvious at presentation died of their primary tumour within 8 months, whereas seven of the 10 patients with occult tumours at presentation are alive 1.5-16.5 years later, and appear cured. [ncbi.nlm.nih.gov]
Meningioma presenting with Cushing's syndrome: An unusual clinical presentation. Ann Neurol 2003;53:138-42. [ijem.in]
Here we present a case of ectopic ACTH syndrome diagnosed with octreotide scan. Case: A 26-year-old male patient presented with moon face, purplish striae, supraclavicular fat pads and proximal muscle weakness. [endocrine-abstracts.org]
Adrenocorticotropic hormone-dependent Cushing syndrome Corticotropin-dependent Cushing syndrome Prevalence: Unknown Inheritance: - Age of onset: - ICD-10: E24.0 OMIM: - UMLS: C0342442 MeSH: - GARD: - MedDRA: - The documents contained in this web site are presented [orpha.net]
Entire Body System
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Chest Discomfort
Other subjective complaints observed included nausea in 2.1% of patients, neck pain in 2.1%, and chest discomfort in 1%. These patients did not go on to develop adverse reactions. [ajnr.org]
Respiratoric
Musculoskeletal
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Neck Pain
Several patients were identified with subjective complaints of nausea, neck pain, or headache, with no sequelae. [ajnr.org]
Face, Head & Neck
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Neck Swelling
Though he developed a gradually progressive left neck swelling postoperatively, he remained otherwise absolutely asymptomatic till last year. [ijem.in]
Urogenital
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Polymenorrhea
Results: Only 6 women (17.6%) had a normal cycle, 9- (26.5%) oligomenorrhea, 12-(35.3) amenorrhea and 7- (20.6%) polymenorrhea. [endocrine-abstracts.org]
Workup
[…] ovarian tumors adrenocorticotropin (ACTH)-independent macronodular adrenocortical hyperplasia (AIMAH) : rare cause of ACTH-independent Cushing syndrome 4 corticotropin-releasing hormone-secreting tumor: very rare hypothalamic tumors ectopic production The workup [radiopaedia.org]
A traditional workup of a patient with ACTH-dependent Cushing's syndrome might include a clinical history, biochemical testing, neuroimaging, and an inferior petrosal sinus sampling (IPSS). [pituitary.org]
Because acute illness activates the HPA axis, resulting in increases in ACTH and cortisol, the laboratory workup for Cushing syndrome should not be performed when subjects are acutely ill. [aippg.net]
[…] updated 4/29/2008. 7.0 7.1 7.2 Gail K Adler, MD, PhD, FAHA, Assistant Professor, Department of Medicine, Division of Endocrinology, Diabetes and Hypertension, Brigham and Women's Hospital, Harvard Medical School Cushing Syndrome: Differential Diagnoses & Workup [physio-pedia.com]
The endocrinologist needs to be aware of the pitfalls of the tests in use, and that these may need repeating over time if the source of ACTH is not found at initial diagnostic workup. REFERENCES 1. Newell-Price J, Bertagna X, Grossman AB, Nieman LK. [scielo.br]
Treatment
As more targeted-approaches are now available, mitotane remains but a remote option for medical treatment in benign CS. [thieme-connect.com]
The primary study hypothesis is that FKBP5 levels are elevated in patients with Cushing's syndrome, and these levels decrease after successful surgical treatment. [clinicaltrials.gov]
A collaboration between MRC Technology and Prof Adrian Clark and Dr Li Chan at the William Harvey Research Institute, Queen Mary University of London has been launched to develop melanocortin receptor type 2 (MC2R) antagonists for the treatment of ACTH-dependent [lifearc.org]
Conclusion: Retinoic acid treatment may alter the clinical presentation of ACTH-dependent CS and consequently delay the diagnosis. [journals.aace.com]
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]
Prognosis
Esthesioneuroblastoma: prognosis and management. Neurosurgery. 1993 ;32: 706 – 714. Google Scholar ISI 9. Benfari, G, Fusconi, M, Ciofalo, A. Radiotherapy alone for local tumour control in esthesioneuroblastoma. [journals.sagepub.com]
The prognosis is worsened in the setting of an unresectable hormone-secreting tumor. [statpearls.com]
[…] features, treatment and survival of ECS-NET patients Materials and methods: Retrospective analysis of data on ECS-NET from 17 centers, obtained by a questionnaire Conference: 13th Annual ENETS conference (2016) Category: Epidemiology/Natural history/Prognosis [enets.org]
Adrenal carcinoma These are very aggressive and the prognosis is poor. Generally, if there are not widespread metastasis, then you should treat the primary tumour surgically. [almostadoctor.co.uk]
Prognosis is favorable if surgery is curative. The rare adrenocortical carcinomas are associated with a 5-year survival rate of 30% or less. [emedicine.medscape.com]
Etiology
Treatment and prognosis Management depends on the specific etiology 6. [radiopaedia.org]
Published: July 11, 2013 By Sherry Boschert, Family Practice News Digital Network SAN FRANCISCO – The size of a pituitary tumor on magnetic resonance imaging in a patient with ACTH-dependent Cushing's syndrome can't differentiate between etiologies, but [pituitary.org]
If the ACTH level is low (20 pg/mL), then the etiology is ACTH-dependent (pituitary adenoma, ectopic source, or CRH secretion). High-dose dexamethasone suppression testing and CRH stimulation test will confirm the etiology. [usmleforum.com]
License: CC BY 3.0 Definition and Etiology of Hypercortisolism The etiology differentiates between an exogenous hypercortisolism, due to long-term treatment with glucocorticosteroids, and an endogenous increased production and secretion of cortisol or [lecturio.com]
Classification & Etiology of Cushing Syndrome Classification & Etiology Frequency (%) ACTH Dependent Cushing Disease 68 Ectopic ACTH syndrome 15 ACTH Independent Adrenal adenoma 9 Adrenal carcinoma 8 Approximately 15 to 30% of the cases of endogenous [clinlabnavigator.com]
Epidemiology
[…] multicenter study on clinicopathologic features, treatment and survival of ECS-NET patients Materials and methods: Retrospective analysis of data on ECS-NET from 17 centers, obtained by a questionnaire Conference: 13th Annual ENETS conference (2016) Category: Epidemiology [enets.org]
Background/Epidemiology/Aetiology Clinical syndrome due to chronic exposure to excess glucocorticoid hormones irrespective of the underlying cause. [dundeemedstudentnotes.wordpress.com]
Biostatistics, Epidemiology and Public Health Unit, Department of Cardiac, Thoracic and Vascular Sciences University Hospital Padua Italy 4. Neuroradiology Unit University Hospital Padua Italy [link.springer.com]
Cushing's syndrome: epidemiology and developments in disease management. Clin Epidemiol 2015; 7:281–293 [Google Scholar] 8. Steffensen C, Bak AM, Rubeck KZ, Jorgensen JO. Epidemiology of Cushing's syndrome. [ajronline.org]
EPIDEMIOLOGY: Cushing syndrome can occur at any age, but usual onset is between 30 and 50 years of age. It is two to three times more common in women than in men. [wikilectures.eu]
Pathophysiology
Study Group of the Italian Society of Endocrinology on the Pathophysiology of the hypothalamic–pituitary–adrenal axis. [link.springer.com]
Understanding Pathophysiology (3rd Ed.). St. Louis, MO: Mosby. Nieman, L.K. (2012). Causes and pathophysiology of Cushing’s syndrome. In A. Lacroix and K.A. Martin (Eds.), UpToDate. Retrieved from cushings-syndrome [wikilectures.eu]
Pecori Giraldi, F, Invitti, C, Cavagnini, F; Study Group of the Italian Society of Endocrinology on the Pathophysiology of the Hypothalamic-Pituitary-Adrenal Axis. [journals.sagepub.com]
Useful For Suggests clinical disorders or settings where the test may be helpful Determining the cause of hypercortisolism and hypocortisolism Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to [mayocliniclabs.com]
Prevention
At the time of discharge, the patient should have a dietary consult and how to prevent osteoporosis. The pharmacist should educate the patient on the prevention of peptic ulcer disease, diabetes, and weight gain. [statpearls.com]
The mass completely involved the sella (preventing pituitary gland identification) although without compromising the pituitary stalk and the optic chiasm ( Figure 2 ). [journals.sagepub.com]
To prevent weight gain, aerobic exercise is good to increase your heart rate. Examples of aerobic exercise include fast walking, jogging, cycling, and swimming. [6] Education on avoiding falls and removing loose rugs and other hazards in the home. [physio-pedia.com]
A false-positive test can be caused by drugs which increase the metabolic breakdown of dexamethasone, preventing its ability to suppress cortisol levels (phenytoin, carbamazepine, and rifampin). [usmleforum.com]
Because glucocorticoids suppress the immune system, they reduce inflammation and are also frequently given to people who have had organ transplants to prevent the immune cells from attacking the transplant. [endocrinediseases.org]