An ectopic Cushing’s syndrome as a cause of severe refractory hypokalemia in the ICU

Mohib, Othmane; Papleux, Emmanuelle; Remmelink, Myriam; Gottignies, Philippe; Bels, David De

doi:10.1080/17843286.2020.1734162

Cited by 3 publications

(3 citation statements)

References 22 publications

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“…The clinical manifestations of hypercortisolism are diverse, and the severity is partly related to the duration of the cortisol increase. [8] Clival tumors are typically uncommon, accounting for 1% of all intracranial tumors. There are many differential diagnoses for clival lesions, including the most common chordoma (40%), meningioma, chondrosarcoma, astrocytoma, craniopharyngioma, germ cell tumors, non-Hodgkin lymphoma, melanoma, metastatic carcinoma, and rarely pituitary adenoma.…”

Section: Discussionmentioning

confidence: 99%

“…[7] Ectopic CS is a very rare disorder of CS caused by an ACTH-secreting tumor outside the pituitary or adrenal gland. [8] It has been reported that ectopic ACTH-producing pituitary adenoma (EAPA) can occur in the sphenoid sinus, cavernous sinus, clivus, and suprasellar region, [9] with EAPA in the clivus region being extremely rare, and merely 6 cases have been reported in the English literature (Table 1). [10][11][12][13][14][15] Furthermore, as summarized in the Table 1, EAPA in the clivus area has unique symptoms, which may lead to misdiagnosis as well as delay in treatment.…”

Section: Introductionmentioning

confidence: 99%

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Cushing syndrome caused by an ectopic ACTH-producing pituitary adenoma of the clivus region: A rare case report and literature review

Tang¹,

Shi²,

Lu³

et al. 2023

Medicine

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Rationale: Ectopic ACTH-producing pituitary adenoma (EAPA) of the clivus region is extraordinarily infrequent condition and merely a few reports have been reported to date. Patient concerns: The patient was a 53-year-old woman who presented with Cushing-like appearances and a soft tissue mass in the clivus region. Diagnoses: The final diagnosis of clivus region EAPA was established by clinical, radiological and histopathological findings. Interventions: The patient underwent gross total clivus tumor resection via transsphenoidal endoscopy. Outcomes: Half a year after surgery, the patient Cushing-like clinical manifestations improved significantly, and urinary free cortisol and serum adrenocorticotropin (ACTH) returned to normal. Lessons: Given the extreme scarcity of these tumors and their unique clinical presentations, it may be possible to misdiagnose and delayed treatment. Accordingly, it is especially crucial to summarize such lesions through our present case and review the literature for their precise diagnosis and the selection of optimal treatment strategies.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Cushing syndrome caused by an ectopic ACTH-producing pituitary adenoma of the clivus region: A rare case report and literature review

Tang¹,

Shi²,

Lu³

et al. 2023

Medicine

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“…The main goal in the EAS treatment approach is curative surgical resection. Medical treatment is used in the following cases: contraindication to surgery or preoperative preparation phase, postoperative hypercortisolism status (relapse/insufficient surgery), CS treatment in non-localizable tumors [21]. Alba et al's algorithmic treatment approach on this issue is up-todate and comprehensive.…”

Section: A: Malignant Tumoral Proliferation Consisting Of Small Cells...mentioning

confidence: 99%

Uncommon presentation of small cell lung carcinoma with ectopic adrenocorticotropic hormone secretion and resistant hypokalemia: A case report

Geçgel,

Alp,

Karpuzoğlu

et al. 2023

Ukr. J. Nephrol. and Dial.

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Paraneoplastic syndromes can serve as initial indicators of malignancy, with small cell lung cancer accounting for 13% of new lung cancer diagnoses. The most prevalent paraneoplastic syndrome associated with small cell lung cancer is inappropriate antidiuretic hormone syndrome, followed by ectopic adrenocorticotropic hormone-mediated Cushing’s syndrome. Cushing’s syndrome manifests as hypercortisolemia and presents with diverse symptoms, including central obesity, plethora, menstrual irregularities, hypertension/diabetes mellitus, ecchymoses, osteoporosis, muscle weakness, virilization/hirsutism, skin atrophy, decreased libido, and infertility. This case report details the uncommon presentation of small cell lung carcinoma manifesting with ectopic adrenocorticotropic hormone secretion (EAS), leading to resistant hypokalemia and rhabdomyolysis. This case emphasizes the importance of considering EAS in severe cases of Cushing's syndrome and highlights the diagnostic and therapeutic challenges associated with this condition.

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Review of the Pathophysiologic and Clinical Aspects of Hypokalemia in Children and Young Adults: an Update

Bamgbola

2022

Curr Treat Options Peds

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This article examines the regulatory function of the skeletal muscle, renal, and adrenergic systems in potassium homeostasis. The pathophysiologic bases of hypokalemia, systematic approach for an early diagnosis, and therapeutic strategy to avert life-threatening complications are highlighted. By promoting skeletal muscle uptake, intense physical exercise (post), severe trauma, and several toxins produce profound hypokalemia. Hypovolemia due to renal and extra-renal fluid losses and ineffective circulation activate secondary aldosteronism causing urinary potassium wasting. In addition to hypokalemic alkalosis, primary aldosteronism causes low-renin hypertension. Non-aldosterone mineralocorticoid activation leading to low-renin and low-aldosterone hypertension occurs in Liddle’s syndrome and apparent mineralocorticoid excess. Although there is enzymatic inhibition of cortisol synthesis in congenital adrenal hyperplasia, precursors of aldosterone produce low-renin hypokalemic hypertension. In addition to the glucocorticoid effect, hypercortisolism activates mineralocorticoid receptors in Cushing’s syndrome. Genetic mutations involving furosemide-sensitive Na + -K + -2Cl − co-transporters and thiazide-sensitive Na + -Cl − transporters result in (non-hypertensive) salt-wasting nephropathy. Proximal and distal renal tubular acidosis is associated with hypokalemia. Eating disorders causing hypokalemia include bulimia, laxative abuse, and diuretic misuse. Low urinary potassium (<15 mmol/day) and/or low urinary chloride (<20 mol/L) suggest a gastrointestinal pathology. Co-morbidity of hypokalemia with chronic pulmonary and cardiovascular diseases may increase the fatality rate.

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An ectopic Cushing’s syndrome as a cause of severe refractory hypokalemia in the ICU

Cited by 3 publications

References 22 publications

Cushing syndrome caused by an ectopic ACTH-producing pituitary adenoma of the clivus region: A rare case report and literature review

Cushing syndrome caused by an ectopic ACTH-producing pituitary adenoma of the clivus region: A rare case report and literature review

Uncommon presentation of small cell lung carcinoma with ectopic adrenocorticotropic hormone secretion and resistant hypokalemia: A case report

Review of the Pathophysiologic and Clinical Aspects of Hypokalemia in Children and Young Adults: an Update

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