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Ectopic ACTH Syndrome

Cushing's Syndrome


Presentation

  • Ectopic ACTH syndrome from small cell carcinoma often presents with hypokalemia, metabolic alkalosis and muscle wasting. This patient presented with Cushingnoid features which is unusual in such cases.[ncbi.nlm.nih.gov]
  • Afterwards, he presented hormonal normalization, normal catecholamines levels, and clinical improvement. Histological tissue analysis confirmed pheochromocytoma.[ncbi.nlm.nih.gov]
  • OBJECTIVE: Expert opinion and a consensus statement on Cushing syndrome (CS) indicate that in a patient with a clinical presentation and biochemical studies consistent with a pituitary etiology, the presence of a pituitary tumor 6 mm is highly suggestive[ncbi.nlm.nih.gov]
  • In this mini-review, we present a patient presenting to our department with a pheochromocytoma as well as Cushing's syndrome due to ectopic ACTH secretion by the pheochromocytoma, and we discuss published cases in the world literature to assess its significance[ncbi.nlm.nih.gov]
  • Small-cell lung cancer presenting as ectopic adrenocorticotropic hormone (ACTH) syndrome associated with hypothyroidism and hypogonadotropic hypogonadism is clinically very rare.[ncbi.nlm.nih.gov]
Respiratory Distress
  • A secondary E. coli infection was superimposed and the patient died of disseminated intravascular coagulation and adult respiratory distress syndrome.[ncbi.nlm.nih.gov]

Workup

  • The diagnostic workup is difficult, can last more than 6 months ( 50% of cases), and the underlying tumour is still frequently not located (12%).[ncbi.nlm.nih.gov]
  • We describe a 34-year-old female patient and discuss the complex diagnostic workup that led to the rare diagnosis of carcinoid of the appendix as the cause of Cushing's syndrome.[ncbi.nlm.nih.gov]
  • EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rapid biochemical control of hypercortisolaemia.[ncbi.nlm.nih.gov]
  • […] 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]
  • Addison Disease Workup. Available online at . Accessed Sept. 20, 2012. (April 2012) Cushing’s Syndrome. National Endocrine and Metabolic Diseases Information Service. Available online at . Accessed on Sept.19 2012. (Updated Dec 12, 2011).[labtestsonline.it]
Enlargement of the Liver
  • A computed tomographic (CT) scan revealed intact pituitary and enlarged adrenal glands. Liver metastases were seen but the primary neoplasm was not found. Treatment with aminoglutethimide and other therapeutic measures were unsuccessful.[ncbi.nlm.nih.gov]

Treatment

  • MTP treatment was started to reduce cortisol production. ACTH levels and cortisol and UFC levels were normalised and the ACTH-producing lung tumour was ablated after MTP treatment.[ncbi.nlm.nih.gov]
  • The treatment consisted of surgical removal of the mass supplemented by radiotherapy. The results showed that after treatment all patients had satisfactory remission.[ncbi.nlm.nih.gov]
  • The patient presented cachexia and developed multiple pulmonary abscesses that also prevented surgical treatment, thus he was treated with percutaneous ethanol injection guided by CT of the left adrenal tumor.[ncbi.nlm.nih.gov]
  • CONCLUSION: Long-term treatment of ectopic ACTH-induced hypercortisolemia by blocking adrenal steroidogenesis is clinically effective and well tolerated.[ncbi.nlm.nih.gov]
  • The key points to increase treatment effect include early detection, localization and resection of primary tumors.[ncbi.nlm.nih.gov]

Prognosis

  • MAIN OUTCOME MEASURES: Etiologies, clinical, biochemical and radiological features, prognosis, and treatment were described.[ncbi.nlm.nih.gov]
  • […] information 1 Department of Endocrinology, The Queen Elizabeth Hospital, Adelaide, Australia. [email protected] Abstract Small cell lung cancer (SCLC) with ectopic adrenocorticotropic hormone (ACTH) syndrome and resultant hypercortisolism carries a poor prognosis[ncbi.nlm.nih.gov]
  • It should be pointed out that the prognosis of bronchial carcinoids is good while that of thymic carcinoids is relatively poor.[ncbi.nlm.nih.gov]
  • These patients have a poor prognosis because SCLC associated with the EAS is more resistant to chemotherapy and the severe hypercortisolism is responsible for a high rate of life-threatening complications during treatment.[ncbi.nlm.nih.gov]
  • Abstract Thymic carcinoid is an important component of the tumor spectrum causing Ectopic ACTH Syndrome (EAS) and usually carries a poor prognosis.[ncbi.nlm.nih.gov]

Etiology

  • However, the 6-mm tumor size cut-off value provided 96% specificity and may be a reasonable threshold to proceed with surgery without the need for IPSS when the biochemical data support a pituitary etiology.[ncbi.nlm.nih.gov]
  • Abstract The etiology of the ectopic ACTH syndrome, associated with certain tumors, is unclear. The ectopic ACTH syndrome was diagnosed in our patient by the characteristic clinical and laboratory findings.[ncbi.nlm.nih.gov]
  • MAIN OUTCOME MEASURES: Etiologies, clinical, biochemical and radiological features, prognosis, and treatment were described.[ncbi.nlm.nih.gov]
  • Treatment and prognosis Management depends on the specific etiology 6.[radiopaedia.org]
  • In 12% the primary etiology was not detected. The rare syndrome of ectopic CRH syndrome (6 published cases) leading to excessive stimulation of the pituitary which became hyperplastic and secreted excessive amounts of ACTH is discussed.[ncbi.nlm.nih.gov]

Epidemiology

  • Although diagnostic evaluation is similar to that in adults, the variation in epidemiology may sway investigations, leading to inappropriate and/or incomplete diagnostic interventions.[ncbi.nlm.nih.gov]
  • 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]
  • Cushing's syndrome: epidemiology and developments in disease management. Clin Epidemiol 2015;7:281-93. [ PubMed ] Díez JJ, Iglesias P. Pharmacological therapy of Cushing's syndrome: drugs and indications.[jtd.amegroups.com]
Sex distribution
Age distribution

Pathophysiology

  • Despite the progress achieved in elucidating the pathophysiology of Cushing's syndrome, clinicians continue to experience diagnostic and therapeutic challenges.[ncbi.nlm.nih.gov]
  • Study group of the Italian Society of Endocrinology on the pathophysiology of the hypothalamic-pituitaryadrenal axis. J Clin Endocrinol Metab. 1999;84:440–8. CrossRef PubMed Google Scholar 29.[link.springer.com]
  • Invitti C, Pecori Giraldi F, Cavagnini F, the Study Group of the Italian Society of Endocrinology on the Pathophysiology of the Hypothalamic-Pituitary-Adrenal Axis.[link.springer.com]
  • We describe a patient with severe hypertension, metabolic alkalosis and hypokalemia as the initial manifestations of an ACTH-secreting small-cell lung carcinoma and review the pathophysiological mechanisms responsible.[jacpjournal.org]

Prevention

  • The patient presented cachexia and developed multiple pulmonary abscesses that also prevented surgical treatment, thus he was treated with percutaneous ethanol injection guided by CT of the left adrenal tumor.[ncbi.nlm.nih.gov]
  • Hypercortisolaemia was controlled with metyrapone and ketoconazole, but high doses of octreotide failed to affect plasma cortisol and urinary 5-hydroxyindole acetic acid (5HIAA) levels, or prevent rapid tumour growth.[ncbi.nlm.nih.gov]
  • Our case indicates that in addition to prophylaxis, the early diagnosis of PCP and the slow correction of hypercortisolemia should be considered in order to prevent an exacerbation due to the reconstitution of the immune function in patients with ectopic[ncbi.nlm.nih.gov]
  • Management of these patients demands an aggressive evaluation to prevent unnecessary adrenalectomy or hypophysectomy and to allow earlier resections before these potentially curable malignancies metastasize.[ncbi.nlm.nih.gov]
  • Prevention: Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable.[uclahealth.org]

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