Symptoma

Ectopic ACTH Syndrome

Cushing's Syndrome

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]

  • Cushingoid

    She received chemotherapy and radiotherapy then and gradually lost her cushingoid appearance. This is the first reported case of clear cell sarcoma of the kidney with ectopic ACTH syndrome. [ncbi.nlm.nih.gov]

  • Leg Edema

    We describe a 63-year-old man with metastatic LCNEC to the left temporomandibular joint (TMJ) who presented with progressive muscle weakness and bilateral lower leg edema for 2 weeks. [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]

    Ten days after the start of ketoconazole, her cortisol level was 51.3 μg/dl (5–25.0); 24-h urine cortisol, 1202 μg/24-h total volume (20–90) and ACTH, 235 pg/ml ( Bilateral pneumonia due to MRSA and respiratory distress occurred due to her immunosuppressive [edmcasereports.com]

  • Rectal Mass

    A 25-year-old black homosexual was noted to be hypertensive, hypokalemic, and to have a rectal mass. Histopathology of the biopsied lesion revealed a mixture of poorly differentiated squamous cell and undifferentiated small cell carcinoma. [ncbi.nlm.nih.gov]

  • Dyspepsia

    Plasma corticotropin-releasing factor was normal, as was β-melanocyte-stimulating hormone and gut hormone profile, except for an elevated gastrin level (this was consistently found, but there was no dyspepsia and gastroscopy was negative) (see table). [pmj.bmj.com]

  • Formication

    The plasma was acidified with 1% (v/v) formic acid and fractioned by Sephadex G-75 gel chromatography. The extracted fractions were evaluated for ACTH and ACTH precursors immunoreactivity. [hormones.gr]

  • Limb Weakness

    A 48 years old man, HIV infected, was admitted with polyuria, polidipsia and lower limb weakness. He had hipertension, crackles in the right hemithorax and linphanedophathy. [imed.pub]

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]

[…] 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]

  • Mediastinal Mass

    For all six patients, the high dose (8 mg) dexamethasone suppression test (HDDST) showed lack of suppression, computed tomography (CT) scanning documented anterior mediastinal masses, and the mediastenal tumors removed were confirmed as ACTH secreting [ncbi.nlm.nih.gov]

    In addition to facilitating tumor location, CT with contrast for mediastinal masses could help surgeons to assess the invasion of tumors and estimate the feasibility of complete resection prior to surgery ( Figure 1B ). [dovepress.com]

  • Anterior Mediastinal Mass

    For all six patients, the high dose (8 mg) dexamethasone suppression test (HDDST) showed lack of suppression, computed tomography (CT) scanning documented anterior mediastinal masses, and the mediastenal tumors removed were confirmed as ACTH secreting [ncbi.nlm.nih.gov]

  • 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]

  • Liver Biopsy

    Liver biopsy confirmed metastatic anorectal carcinoma, and immunohistochemical stains of the rectal biopsy were positive for ACTH and neuron-specific enolase. [ncbi.nlm.nih.gov]

    Liver biopsies were also positive for the neuroendocrine markers CgA, synaptophysin, neuron-specific enolase, and CD56. Cancer antigen 19-9 levels were very high at 2027.9 U/mL (reference range,  35 U/mL). [consultant360.com]

    CT-guided liver biopsy revealed tumour cells of neuroendocrine origin consistent with carcinoid tumour, and urinary 5-hydroxyindole-acetic acid (5HIAA) was high (see table). [pmj.bmj.com]

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]

MAIN OUTCOME MEASURES: Etiologies, clinical, biochemical and radiological features, prognosis, and treatment were described. [ncbi.nlm.nih.gov]

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]

Treatment and prognosis Management depends on the specific etiology 6. [radiopaedia.org]

Furthermore, if the lung nodule is a benign lung hemangioma, we recommend that physicians not exclude it as the etiology of Cushing syndrome. [kjim.org]

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]

Ectopic CRH secretion leading to increased ACTH secretion comprises a very rare group of cases of Cushing syndrome. [4] Epidemiology Identifying the exact incidence of Cushing syndrome is challenging because patients can present with a wide spectrum from [emedicine.medscape.com]

One hundred years after “carcinoid”: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26(18):3063–3072. 7. Travis W, Brambilla E, Müller-Hermelink H, Harris C, editors. [dovepress.com]

Despite the progress achieved in elucidating the pathophysiology of Cushing's syndrome, clinicians continue to experience diagnostic and therapeutic challenges. [ncbi.nlm.nih.gov]

This Second Edition has been thoroughly updated with the latest information on pathophysiology, treatment, and drug dosing. New chapters cover obesity, vitamin D deficiency, autoimmune polyendocrine syndromes, and endocrine disorders in HIV/AIDS. [books.google.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]

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]

Prevention: Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable. [uclahealth.org]