Spontaneous remission of ectopic Cushing's syndrome due to pheochromocytoma: a case report

1996 ◽  
Vol 135 (4) ◽  
pp. 440-443 ◽  
Author(s):  
Keh-Chuan Loh ◽  
Rekha Gupta ◽  
Allan H Shlossberg
Keyword(s):  
Case Report ◽  
Cushing’S Syndrome ◽  
Spontaneous Remission ◽  
Cushing's Syndrome ◽  
Young Female ◽  
Cell Hyperplasia ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome ◽  
Ectopic Acth Production ◽  
Biochemical Remission

Loh K-C, Gupta R, Shlossberg AH. Spontaneous remission of ectopic Cushing's syndrome due to pheochromocytoma: a case report. Eur J Endocrinol 1996;135:440–3. ISSN 0804–4643 A young female adult with Cushing's syndrome arising from ectopic production of corticotropin (ACTH) from an adrenal pheochromocytoma showed spontaneous clinical and biochemical remission of hypercortisolism after a brief period of ketoconazole administration. Despite continued remission of the hypercortisolism over the next 18 months, there was progressive catecholamine hypersecretion with significant morbidity as a result of the pheochromocytoma. Surgical resection of the left adrenal gland revealed a pheochromocytoma showing focal cytoplasmic immunostaining for ACTH and marked diffuse compact cell hyperplasia in the adrenocortical tissue. To our knowledge this is the first reported case of spontaneous clinical and biochemical remission of ectopic ACTH production from a pheochromocytoma. The pathogenesis of the remission remains unknown. Keh-Chuan Loh, Division of Endocrinology and Metabolism, Dalhousie University, 5303 Morris Street, Halifax, Nova Scotia B3J 1B6, Canada

scholarly journals Fluconazole and acetazolamide in the treatment of ectopic Cushing's syndrome with severe metabolic alkalosis

2015 ◽  
Vol 2015 ◽  
Author(s):  
Verena Schwetz ◽  
Felix Aberer ◽  
Claudia Stiegler ◽  
Thomas R Pieber ◽  
Barbara Obermayer-Pietsch ◽  
...  
Keyword(s):  
Case Report ◽  
Surgical Resection ◽  
Cushing’S Syndrome ◽  
Metabolic Alkalosis ◽  
Cushing's Syndrome ◽  
List Type ◽  
Ectopic Acth ◽  
Ectopic Cushing’S Syndrome ◽  
Ectopic Acth Production ◽  
Learning Points

Summary Cushing's syndrome (CS) due to ectopic ACTH production accounts for about 10% of all types of CS and is frequently associated with metabolic alkalosis. Treatment of CS involves surgical resection and/or medical therapy to control hypercortisolism. We present the case of an 80-year-old woman affected by CS due to an unknown cause. The patient had severe metabolic alkalosis with refractory hypokalemia. To treat the underlying CS, fluconazole was initiated due to unavailability of ketoconazole. In spite of markedly decreasing cortisol levels, metabolic alkalosis persisted. Treatment of metabolic alkalosis with acetazolamide was thus initiated and pH levels successfully lowered. This case report shows that hypercortisolism can be effectively treated with fluconazole in cases where ketoconazole is unavailable or not tolerated and that persistent severe metabolic alkalosis caused by glucocorticoid excess can be safely and successfully treated with acetazolamide. Learning points Hypercortisolism can be effectively treated with fluconazole where ketoconazole is unavailable or not tolerated. Glucocorticoid excess can cause severe metabolic alkalosis. Persistent severe metabolic alkalosis can be safely and successfully treated with acetazolamide.

scholarly journals Cushing’s Syndrome, Cortisol, and Cognitive Competency: A Case Report

2017 ◽  
Vol 10 (1) ◽  
pp. 325-327
Author(s):  
Neil Oronsky ◽  
Bennett Thilagar ◽  
Carolyn M. Ray ◽  
Scott Caroen ◽  
Michelle M.C. Lybeck ◽  
...  
Keyword(s):  
Case Report ◽  
Antibiotic Treatment ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Care Planning ◽  
Advanced Care Planning ◽  
Ectopic Acth ◽  
Neuropsychiatric Complications ◽  
Ectopic Acth Production ◽  
Cognitive Competency

Glucocorticoids are associated with immunosuppression and neuropsychiatric complications. We describe the case of a carcinoid patient with Cushing’s syndrome (CS) and neurocognitive impairment due to ectopic ACTH production who developed sepsis and died because of his family’s decision to withdraw antibiotic treatment. This report is presented to illustrate the importance of advanced-care planning in patients with CS.

scholarly journals Rapid control of ectopic Cushing’s syndrome during the COVID-19 pandemic in a patient with chronic hypokalaemia

2021 ◽  
Vol 2021 ◽  
Author(s):  
Ziadoon Faisal ◽  
Miguel Debono
Keyword(s):  
Type 2 Diabetes ◽  
Case Report ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Alternative Methods ◽  
Ectopic Acth Syndrome ◽  
High Dose ◽  
List Type ◽  
Ectopic Acth

Summary In this case report, we describe the management of a patient who was admitted with an ectopic ACTH syndrome during the COVID pandemic with new-onset type 2 diabetes, neutrophilia and unexplained hypokalaemia. These three findings when combined should alert physicians to the potential presence of Cushing’s syndrome (CS). On admission, a quick diagnosis of CS was made based on clinical and biochemical features and the patient was treated urgently using high dose oral metyrapone thus allowing delays in surgery and rapidly improving the patient’s clinical condition. This resulted in the treatment of hyperglycaemia, hypokalaemia and hypertension reducing cardiovascular risk and likely risk for infection. Observing COVID-19 pandemic international guidelines to treat patients with CS has shown to be effective and offers endocrinologists an option to manage these patients adequately in difficult times. Learning points This case report highlights the importance of having a low threshold for suspicion and investigation for Cushing’s syndrome in a patient with neutrophilia and hypokalaemia, recently diagnosed with type 2 diabetes especially in someone with catabolic features of the disease irrespective of losing weight. It also supports the use of alternative methods of approaching the diagnosis and treatment of Cushing’s syndrome during a pandemic as indicated by international protocols designed specifically for managing this condition during Covid-19.

Ectopic cushing's syndrome secondary to acinic cell carcinoma of the parotida gland: A case-report

2021 ◽  
Author(s):  
Sara Pinheiro ◽  
Juliana Filipe ◽  
Pedro Meireles ◽  
Branco Sara Castelo ◽  
Miguel Rito ◽  
...  
Keyword(s):  
Case Report ◽  
Cell Carcinoma ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Acinic Cell Carcinoma ◽  
Ectopic Cushing’S Syndrome ◽  
Acinic Cell

Ectopic ACTH-secreting tumor of the thymus revealed by a Cushing’s syndrome: case report and review of literature

2017 ◽  
Vol 34 (1) ◽  
pp. 65-68
Author(s):  
Zied Chaari ◽  
Hazem Zribi ◽  
Sonia Ouerghi ◽  
Mohamed-Sadok Boudaya ◽  
Tarek Kilani ◽  
...  
Keyword(s):  
Case Report ◽  
Cushing’S Syndrome ◽  
Cushing's Syndrome ◽  
Review Of Literature ◽  
Ectopic Acth ◽  
Acth Secreting

scholarly journals Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma

2013 ◽  
Vol 2013 ◽  
Author(s):  
F Serra ◽  
S Duarte ◽  
S Abreu ◽  
C Marques ◽  
J Cassis ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Microscopic Analysis ◽  
Cushing's Syndrome ◽  
Ectopic Acth Syndrome ◽  
List Type ◽  
Rare Tumour ◽  
Ectopic Acth ◽  
Ectopic Acth Production ◽  
The Right

Summary Ectopic secretion of ACTH is an infrequent cause of Cushing's syndrome. We report a case of ectopic ACTH syndrome caused by a nasal paraganglioma, a 68-year-old female with clinical features of Cushing's syndrome, serious hypokalaemia and a right paranasal sinus' lesion. Cranial magnetic resonance image showed a 46-mm mass on the right paranasal sinuses. Endocrinological investigation confirmed the diagnosis of ectopic ACTH production. Resection of the tumour normalised ACTH and cortisol secretion. The tumour was found to be a paraganglioma through microscopic analysis. On follow-up 3 months later, the patient showed nearly complete clinical recovery. Ectopic ACTH syndrome due to nasal paraganglioma is extremely uncommon, as only two other cases have been discussed in the literature. Learning points Ectopic Cushing's syndrome accounts for 10% of Cushing's syndrome etiologies. Most paraganglioma of the head and neck are not hormonally active. Nasal paraganglioma, especially ACTH producing, is a very rare tumour.

scholarly journals A rare case of duodenal carcinoid presenting as ectopic Cushing’s syndrome

2019 ◽  
Vol 6 (3) ◽  
pp. 959
Author(s):  
Siddharth Pugalendhi ◽  
Tarun Kumar Dutta ◽  
Dhivya . ◽  
Kiran Yadav
Keyword(s):  
Neuroendocrine Tumors ◽  
Cushing’S Syndrome ◽  
Cushing Syndrome ◽  
Somatostatin Analogues ◽  
Cushing's Syndrome ◽  
High Dose ◽  
Ectopic Acth ◽  
High Dose Dexamethasone ◽  
Ectopic Acth Production ◽  
Duodenal Carcinoid

ACTH-dependent Cushing syndrome (CS) due to an ectopic source is responsible for approximately 10-15% cases of Cushing’s syndrome. It is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Many a times ectopic ACTH production is difficult to manage, and identification of the source may take many years.  Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogues or bilateral adrenalectomy in refractory cases and if the source remains unidentified. Here, we report a case who presented with features of Cushing’s syndrome which eventually through workup led us to a diagnosis of duodenal carcinoid producing ectopic ACTH which is extremely rare and was successfully treated.

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