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ACTH-Independent Cushing Syndrome

Cushing's syndrome


Presentation

  • Six of 10 carcinoma patients had metastases at presentation. Two adenomas were seen within a myelolipoma, which was recognized at both CT and MR imaging due to its fat content, and two adenomas were of uncertain malignant potential.[ncbi.nlm.nih.gov]
  • Presented as an education exhibit at the 2002 RSNA scientific assembly. Received April 2, 2003; revision requested May 7 and received June 12; accepted June 13. All authors have no financial relationships to disclose.[vrad.com]
  • These patients usually present at an older age with long standing symptoms.[healio.com]
  • We report a case of a 57-year-old woman who presented with signs and symptoms of Cushing's syndrome who was determined to have AIBMAH. The patient was successfully treated by bilateral laparoscopic adrenalectomy.[journals.lww.com]
  • AIMAH usually presents with bilateral lesions that are larger than 10 mm and it is more frequent in women.[colombiamedica.univalle.edu.co]
Camping
  • Little is known about cAMP signaling in adrenal lesions associated with ACTH-independent Cushing syndrome (AICS) that do not harbor mutations in known genes. OBJECTIVE: We assessed the cAMP-signaling pathway by enzymatic and molecular studies.[ncbi.nlm.nih.gov]
  • In this Review, we discuss Cushing syndrome and its linkage to dysregulated cAMP–PKA signalling, with a focus on genetic findings in the past few years.[nature.com]
  • MEN1 (menin をコード)、AIP (aryl-hydrocarbon receptor-interacting protein をコード)、PRKAR1A ( cAMP-dependent protein kinase type I alpha regulatory subunit をコード)、CDKN1B (cyclin-dependent kinase inhibitor 1B、 p27 Kip1 としても知られる)における生殖細胞系列の変異は、家族性の下垂体腺腫において見つかっている[genome.jp]
Difficulty Climbing Stairs
  • . - Irregular menses and hirsutism - Progressive proximal muscle weakness, patients may have difficulty climbing stairs, getting out of a low chair, and raising their arms. - Psychological problems (depression, cognitive dysfunction, and emotional lability[aippg.net]
Dyspepsia
  • The clinical features are those of glucocorticoid excess: Symptoms: Weight gain Change of appearance Depression Insomnia Amenorrhoea / oligomenorrhoea Poor libido Thin skin / easy bruising Hair growth Acne Slow growth in children Back pain Polyuria Dyspepsia[almostadoctor.co.uk]
Chvostek Sign
  • The physical exam revealed a positive Chvostek sign, facio-troncular obesity, reddish-purple striae and grade I hypertension. Biochemical assessment revealed hypocalcaemia (5.75 mg/dl) and diabetes mellitus.[endocrine-abstracts.org]
Severe Osteoporosis
  • Sometimes asthenic habitus, with short stature, severe osteoporosis, muscle and skin wasting. Near-normal 24-hr UFC, or sometimes cyclical hypercortisolism.[bigtomato.org]
Neglect
  • This may occur in patients on steroids who stop taking their glucocorticoids or neglect to increase their steroids during an acute illness.[aippg.net]
Uremia
  • Obesity, depression, alcoholism, high estrogen states, and uremia can cause false positive results. Chronic renal failure and liver failure can cause false negative results.[clinlabnavigator.com]

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]
  • Diagnostic workup is necessary perioperatively as well. Usually hydrocortisone 100 mg is given IM or IV preoperatively and q6 hours postoperatively until oral supplements are tolerated.[barnardhealth.us]
  • 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

  • […] the treatment of ACTH independent adrenal Cushing's Syndrome.[clinicaltrials.gov]
  • 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]
  • TREATMENT: Treatment options vary and are dependent upon the underlying cause of the hypercortisolism. Therefore differentiation of the cause is essential for effective treatment.[wikilectures.eu]
  • These drugs are not usually effective as long-term treatment and are used mainly for preparation for surgery or as adjunctive treatment after surgery or pituitary radiotherapy.[patient.info]
  • See also Cushing's diagnosis and treatment.[bigtomato.org]

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]
  • […] children, tumors are usually pheochromocytoma, neuroblastoma, thymic or pancreatic endocrine neoplasm Associated with hypokalemic alkalosis, high urinary excretion of free cortisol, skin pigmentation, edema and severe diabetes mellitus Usually poor prognosis[pathologyoutlines.com]
  • The prognosis is guardedly favourable with surgery. The rare adrenocortical carcinomas have a five-year survival rate of 30% or less.[patient.info]
  • Untreated Cushing associated with high risk of mortality, poor prognosis seen in ectopic ACTH syndrome and adrenocortical carcinoma Basis for recommendation Nieman LK, Biller BM, Findling JW, et al.[hopkinsguides.com]

Etiology

  • Jing-Xia Guo Qiao-Hua Ren Bu-Lang Gao Abstract Purpose: To evaluate the value of carbon dioxide combining power/potassium (CO 2 CP/K) ratio in etiological diagnosis of Cushing’s syndrome.[currentscience.org]
  • Treatment and prognosis Management depends on the specific etiology 6.[radiopaedia.org]
  • Case Reports: PDF Only Abstract : ACTH-independent Cushing's syndrome secondary to ACTH-independent bilateral macronodular adrenocortical hyperplasia (AIBMAH) is an uncommon disorder, and in most cases the precise etiology has not been determined.[journals.lww.com]
  • Definition Cushing’s syndrome is a condition which is due to prolonged exposure of the body tissue to excess cortisol (glucocorticoid hormone) Etiology Adrenocorticotropic Hormone (ACTH)-Independent Cushing's Syndrome Endogenous ACTH-Independent Cushing[specialty.mims.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

  • 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]
  • Epidemiology [ 1 ] Incidence of Cushing's syndrome is in the region of 10 to 15 people per million, with a higher incidence in people with diabetes, obesity, hypertension or osteoporosis. [ 4 ] In obese patients with type 2 diabetes, especially those[patient.info]
  • […] intracranial pressure and brain herniation Elimination disorders Embryogenesis Emotional and behavioral disorders in children and adolescents Endarterectomy Endometrial cancer Endometriosis Endophthalmitis Environmental injuries Enzymes and biocatalysis Epidemiology[amboss.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • […] changes from granular to homogenous, due to accumulation of intermediate filaments Adrenal gland changes: exogenous cortisol causes low ACTH, which causes atrophy of fasciculata and reticularis, not glomerulosa, in residual or opposite adrenal gland Pathophysiology[pathologyoutlines.com]
  • A recent pathophysiological study showed that cortisol production was regulated by aberrant membrane receptors and by corticotropin produced within the adrenocortical tissue.[ekjm.org]
  • Molecular analysis of the cyclic AMP-dependent protein kinase A (PKA) regulatory subunit 1A ( PRKAR1A ) gene in patients with Carney complex and primary pigmented nodular adrenocortical disease (PPNAD) reveals novel mutations and clues for pathophysiology[nature.com]
  • Pathophysiology: Endogenous glucocorticoid overproduction or hypercortisolism that is independent of adrenocorticotropic hormone (ACTH) usually is due to a primary adrenocortical neoplasm.[aippg.net]

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]
  • 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]
  • […] not restriction) 17 TREATMENT OF THE CHRONIC ADRENOCORTICAL INSUFFICIENCY GENERAL MEASURES –PREVENTION OF ADRENAL CRISIS The essential elements are patient education and increased glucocorticoid dosages during illness![slidepptx.com]

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