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]
Here we report three cases of undiagnosed bronchogenic carcinoma who presented with EAS within a period of 12 months. Case 1 67-year-old lady, smoker, presented with severe proximal myopathy of 4 week duration. Clinically she appeared cushingoid. [endocrine-abstracts.org]
Respiratoric
- 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]
Gastrointestinal
- 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]
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]
[…] 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 http://emedicine.medscape.com/article/116467-workup. Accessed Sept. 20, 2012. (April 2012) Cushing’s Syndrome. National Endocrine and Metabolic Diseases Information Service. [labtestsonline.it]
X-Ray
- 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]
Ultrasound
- 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]
Biopsy
- 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]
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]
Prognosis
MAIN OUTCOME MEASURES: Etiologies, clinical, biochemical and radiological features, prognosis, and treatment were described. [ncbi.nlm.nih.gov]
(Outcomes/Resolutions) The prognosis of Ectopic Cushing Syndrome is considered to be good, with a full recovery expected after removal of the tumor The prognosis is also dependent upon the severity of the cancer There also remains the possibility that [dovemed.com]
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]
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]
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]
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]
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]
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: 3063-3072. [pubmed] [ 6 ].Jensen RT, Cadiot G, Brandi ML, et al. [npplweb.com]
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]
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]
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]
Prevention: Prompt treatment of tumors may reduce the risk in some cases. Many cases are not preventable. [uclahealth.org]