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ACTH-Dependent Cushing Syndrome

ACTH-Dependent CS


Presentation

  • 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]
  • 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]
  • Adrenal myelolipoma(s) as presenting manifestation of subclinical Cushing’s disease (eutopic ACTH-dependent Cushing’s syndrome) Posted on August 16, 2017 by MaryO Partha Pratim Chakraborty 1, Shinjan Patra 1, Sugata Narayan Biswas 1, Rana Bhattacharjee[cushieblog.com]
  • Our patient presented typical clinical features of CS after three infusions of combined therapy, high serum and daily urinary free cortisol, and high serum ACTH levels.[ncbi.nlm.nih.gov]
  • He first presented to our endocrine unit in December 2011 for evaluation. Results: The patient was in good general health and alert. His medication included atorvastatin 20 mg daily. There were no clinical signs of hypercortisolism.[thieme-connect.com]
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]
Neck Pain
  • Several patients were identified with subjective complaints of nausea, neck pain, or headache, with no sequelae.[ajnr.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]

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]
  • Conclusion: Correct interpretation of the initial clinical presentation can help in the proper diagnosis and treatment of ACTH-dependent Cushing’s syndrome. References Bertagna X, Guignat L, Groussin L, Bertherat J. Cushing's disease.[ejcrim.com]
  • Further treatment depends on the cause: Cushing’s disease – pituitary dependent hyperadrenalism. Trans-sphenoidal removal of the tumour is the treatment of choice.[almostadoctor.co.uk]
  • Make the best clinical decisions with an enhanced emphasis on evidence-based practice and expert opinions on treatment strategies. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography.[books.google.ro]

Prognosis

  • 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]
  • Treatment and prognosis Management depends on the specific etiology 6.[radiopaedia.org]
  • Patients with ectopic ACTH syndrome caused by occult neuroendocrine tumors have a more favorable prognosis than cases caused by small-cell lung cancer, medullary carcinoma of the thyroid, or gastrinoma. 2 It is regrettable that the patient presented in[mdmag.com]
  • The prognosis is guardedly favourable with surgery. The rare adrenocortical carcinomas have a five-year survival rate of 30% or less.[patient.info]
  • Prognosis Untreated Cushing’s syndrome is often fatal, with most deaths occurring from cardiovascular/thromboembolic or bacterial/fungal infections.[clinicaladvisor.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

  • 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]
  • 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]
  • Epidemiology Prevalence 5–6/100.000 Most often women in the 3rd and 4th decade of life Causes (Etiology) of Cushing's Syndrome ACTH-dependent Cushing's Syndrome (Overproduction of ACTH): Pituitary adenoma (Cushing's disease) Paraneoplastic ACTH production[urology-textbook.com]
  • Morbidity and mortality in Cushing's disease: an epidemiological approach. Clin Endocrinol (Oxf). 1994;40(4):479-84.[hopkinsguides.com]
Sex distribution
Age distribution

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]
  • 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[mayomedicallaboratories.com]
  • Epidemiology, pathophysiology and treatment. CNS Drugs. 2001;15(5):361-73. Colao A, Faggiano A, Pivonello R, et al. Inferior petrosal sinus sampling in the differential diagnosis of Cushing’s syndrome: results of an Italian multicenter study.[rarediseases.org]
  • The pathophysiologic roles of inter-leukin-6 in human disease. Ann Intern Med . 1998;128:127–37. 15. Cushing H. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism).[aafp.org]

Prevention

  • 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]
  • 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]
  • Many cases are not preventable. Cushing syndrome - ectopic; Ectopic ACTH syndrome Nieman LK, Biller BM, Findling JW, et al. Treatment of Cushing's syndrome: an Endocrine Society clinical practice guideline.[medlineplus.gov]
  • Porphyrias Portal hypertension Postmortem skin changes Postpartum hemorrhage Postpartum period Poststreptococcal glomerulonephritis Precancerous skin lesions Pregnancy Pregnancy loss Prenatal and postnatal physiology Prenatal care Preterm labor and birth Preventive[amboss.com]
  • Pituitary irradiation before bilateral adrenalectomy may prevent Nelson’s syndrome.[clinicaladvisor.com]

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