scholarly journals Severe Cushing’s syndrome and bilateral pulmonary nodules: beyond ectopic ACTH

2017 ◽  
Vol 2017 ◽  
Author(s):  
Carlos Tavares Bello ◽  
Emma van der Poest Clement ◽  
Richard Feelders
Keyword(s):  
Cushing’S Syndrome ◽  
Prolonged Exposure ◽  
Opportunistic Infections ◽  
Cushing's Syndrome ◽  
List Type ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Thrombotic Risk ◽  
Ectopic Acth Secretion ◽  
Increased Risk

Summary Cushing’s syndrome is a rare disease that results from prolonged exposure to supraphysiological levels of glucocorticoids. Severe and rapidly progressive cases are often, but not exclusively, attributable to ectopic ACTH secretion. Extreme hypercortisolism usually has florid metabolic consequences and is associated with an increased infectious and thrombotic risk. The authors report on a case of a 51-year-old male that presented with severe Cushing’s syndrome secondary to an ACTH-secreting pituitary macroadenoma, whose diagnostic workup was affected by concurrent subclinical multifocal pulmonary infectious nodules. The case is noteworthy for the atypically severe presentation of Cushing’s disease, and it should remind the clinician of the possible infectious and thrombotic complications associated with Cushing’s syndrome. Learning points: Severe Cushing’s syndrome is not always caused by ectopic ACTH secretion. Hypercortisolism is a state of immunosuppression, being associated with an increased risk for opportunistic infections. Infectious pulmonary infiltrates may lead to imaging diagnostic dilemmas when investigating a suspected ectopic ACTH secretion. Cushing’s syndrome carries an increased thromboembolic risk that may even persist after successful surgical management. Antibiotic and venous thromboembolism prophylaxis should be considered in every patient with severe Cushing’s syndrome.

scholarly journals A rare cause of severe Cushing’s syndrome

2020 ◽  
Vol 2020 ◽  
Author(s):  
Shamaila Zaman ◽  
Bijal Patel ◽  
Paul Glynne ◽  
Mark Vanderpump ◽  
Ali Alsafi ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Primary Tumour ◽  
Serum Cortisol ◽  
Cushing's Syndrome ◽  
List Type ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Baseline Serum ◽  
Ectopic Acth Secretion ◽  
Alpha Blockade

Summary Ectopic adrenocorticotropic hormone (ACTH) production is an uncommon cause of Cushing’s syndrome and, rarely, the source can be a phaeochromocytoma. A 55-year-old man presented following an episode of presumed gastroenteritis with vomiting and general malaise. Further episodes of diarrhoea, joint pains and palpitations followed. On examination, he was hypertensive with no clinical features to suggest hypercortisolaemia. He was subsequently found to have raised plasma normetanephrines of 3.98 nmol/L (NR <0.71) and metanephrines of 0.69 nmol/L (NR <0.36). An adrenal CT showed a 3.8 cm right adrenal nodule, which was not MIBG-avid but was clinically and biochemically consistent with a phaeochromocytoma. He was started on alpha blockade and referred for right adrenalectomy. Four weeks later, on the day of admission for adrenalectomy, profound hypokalaemia was noted (serum potassium 2.0 mmol/L) with non-specific ST-segment ECG changes. He was also diagnosed with new-onset diabetes mellitus (capillary blood glucose of 28 mmol/L). He reported to have gained weight and his skin had become darker over the course of the last 4 weeks. Given these findings, he underwent overnight dexamethasone suppression testing, which showed a non-suppressed serum cortisol of 1099 nmol/L. Baseline serum ACTH was 273 ng/L. A preliminary diagnosis of ectopic ACTH secretion from the known right-sided phaeochromocytoma was made and he was started on metyrapone and insulin. Surgery was postponed for 4 weeks. Following uncomplicated laparoscopic adrenalectomy, the patient recovered with full resolution of symptoms. Learning points: Phaeochromocytomas are a rare source of ectopic ACTH secretion. A high clinical index of suspicion is therefore required to make the diagnosis. Ectopic ACTH secretion from a phaeochromocytoma can rapidly progress to severe Cushing’s syndrome, thus complicating tumour removal. Removal of the primary tumour often leads to full recovery. The limited literature suggests that the presence of ectopic Cushing’s syndrome does not appear to have any long-term prognostic implications.

scholarly journals ACTH-Producing Thymic Carcinoid: A Rare Cause of Cushing’s Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A990-A990
Author(s):  
Lisette Patricia Rodriguez ◽  
Wende Michele Kozlow
Keyword(s):  
Cushing’S Syndrome ◽  
Mediastinal Mass ◽  
Cushing's Syndrome ◽  
Anterior Mediastinal Mass ◽  
Thymic Carcinoid ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
History Of

Abstract Background: Thymic carcinoids are rare neoplasms that account for less than 5% of all thymic tumors. Approximately 25% of these tumors will result in Cushing’s syndrome due to ectopic ACTH secretion. These tumors can also be associated with MEN1 syndrome. This is a case report of a patient with history of macroprolactinoma now presenting with Cushing’s syndrome due to ectopic ACTH production from a thymic carcinoid tumor. Clinical Case: This is a 57 year old male with history of pituitary macroprolactinoma diagnosed in 2011, now status post transsphenoidal resection and external beam radiation therapy, with persistent hyperprolactinemia on cabergoline, who presented to our clinic for a routine follow up visit. Patient had already developed secondary hypogonadism and secondary hypothyroidism as a consequence of treatment for the macroprolactinoma. He complained of worsening fatigue and weight gain ongoing for several months. Laboratory studies revealed an hemoglobin A1c of 8.3% (nl &lt; 5.7%), TSH 0.24 MIU/L (0.4-4.5 MIU/L), free T4 1.2 ng/dL (0.8-1.8 ng/dL), 8 AM cortisol 31.4 mcg/dL (4-22 mcg/dL), ACTH 185 pg/mL (6-50 pg/dL), prolactin 29.6 ng/mL (2-18 ng/mL), IGF-1 88 ng/mL (50-317 ng/mL). Follow up labs confirmed cushings syndrome: cortisol AM-DST 36.4 mcg/dL (&lt; 2 mcg/dL), free urinary cortisol 291.9 mcg/24h (2-50 mcg/24h). Pituitary MRI showed empty sella turcica. Cortisol after an 8 mg DST 32.5 mcg/dL (&lt; 5 mcg/dL). CT chest, abdomen and pelvis revealed an heterogeneously enhancing solid anterior mediastinal mass measuring 4.9 x 3.1 x 4.3 cm. Whole body OctreoScan showed a markedly hyperintense large mass adjacent to the right heart border measuring 47 x 32 mm. He was referred to cardiothoracic surgery and underwent a right video-assisted thoracic surgery with resection of the anterior mediastinal mass. Pathology revealed a thymic well-differentiated neuroendocrine tumor with strong cytoplasmic staining for ACTH. It was also positive for OSCAR, Cam5.2, synaptophysin, CD56, and S100. Ki67 stain was positive in fewer than 1% of tumor cells. Final diagnosis was carcinoid tumor. Conclusion: Cushing’s syndrome secondary to ectopic ACTH secretion from a thymic carcinoid is rare. The presence of two MEN1-associated tumors in this patient, macroprolactinoma and thymic carcinoid, is highly suggestive of a clinical diagnosis of MEN 1.

Selective Venous Sampling to Differentiate Ectopic ACTH Secretion from Pituitary Cushing's Syndrome

1977 ◽  
Vol 296 (15) ◽  
pp. 861-862 ◽  
Author(s):  
Dominic F. Corrigan ◽  
Marcus Schaaf ◽  
Robert A. Whaley ◽  
Carol L. Czerwinski ◽  
Jerry M. Earll
Keyword(s):  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Venous Sampling ◽  
Acth Secretion ◽  
Ectopic Acth ◽  
Ectopic Acth Secretion ◽  
Selective Venous Sampling
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