Two types of ectopic Cushing syndrome or a continuum? Review

Araujo‐Castro, Marta; Azpiroz, Monica Marazuela

doi:10.1007/s11102-018-0894-2

Cited by 15 publications

(9 citation statements)

References 82 publications

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“…It should be noted that in our series no cases of ECS of non-lung origin were reported. Although less frequently, ECS is also associated with pancreatic neuroendocrine tumours (NETs), pheohcoromcytomas, thymic carcinoids/carcinomas or medullary thyroid carcinomas ( 2 , 14 ).…”

Section: Discussionmentioning

confidence: 99%

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Ectopic Cushing’s syndrome: clinical, diagnostic, treatment and follow-up outcomes of 12 cases of lung ectopic ACTH

Fernández

Montenegro

Torrecilla

et al. 2023

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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Summary Ectopic Cushing’s syndrome (ECS) is a rare disease associated with significant comorbidity. Among the causes of Cushing's syndrome, adrenocorticotropic hormone-producing extrapituitary tumours are rarely reported. This low frequency makes it difficult for the physician to acquire experience in its management. In this report, we aimed to describe the clinical presentation, diagnostic approach and treatment modalities of 12 patients with ECS treated in a single tertiarycentre over a 17-year period. Although they can appear in different locations through the neuroendocrine system, lung tumours are the most frequently reported, as it occurs in our series. They can show different levels of aggressiveness and mild to severe clinical course. Therefore, distinguishing Cushing's disease can be challenging and sometimes requires more specific techniques such as invasive tests or no conventional imaging. Treatment includes controlling both hypercortisolism and neoplastic disease, and multidisciplinary management is recommended. Learning points Ectopic Cushing's syndrome (ECS) accounts for 15% of endogenous Cushing's syndromes. Its infrequency implies that both diagnosis and treatment can be a challenge for clinicians without experience in its management. The most common location is the lung. Although older series reported small cell lung carcinoma (SCLC) as the main ECS-producing tumour, currently most cases are attributed to lung carcinoids. Low-grade tumours (lung carcinoids) present themselves with a more subtle and gradual hypercortisolism, and clinically this can be difficult to differentiate from hypercortisolism due to CD. In contrast, high-grade tumours (SCLC) show severe hypercortisolism with rapid evolution. The diagnostic approach is complex especially when the tumour is not previously known and the clinical presentation is subtle. Functional tests are mandatory in these cases, and nuclear medicine imaging can help when conventional imaging tests fail to identify the tumour. ECS treatment includes a wide variety of modalities oriented to treat both the excess of cortisol and the tumour itself. The tumour prognosis depends fundamentally on the type of adrenocorticotropic hormone-secreting tumour. Expert and multidisciplinary team is essential for successfully treating these complex and ill patients.

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Section: Discussionmentioning

confidence: 99%

“…Lung carcinoids typically present in a more subtle clinical manner in contrast with SCLC. This gradual development of the clinical syndrome is associated with more subtle biochemistry and leads to considerable challenges in distinguishing this ECS form from pituitary tumours causing Cushing’s disease (CD) ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

Ectopic Cushing’s syndrome: clinical, diagnostic, treatment and follow-up outcomes of 12 cases of lung ectopic ACTH

Fernández

Montenegro

Torrecilla

et al. 2023

Endocrinology, Diabetes &Amp; Metabolism Case Reports

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“…As compared to patients with pituitary corticotroph adenoma (Cushing's disease), patients with ectopic Cushing's syndrome frequently have different clinical features. In these forms, higher cortisol levels and a more sudden increase explain the rapid progression of symptoms, sometimes fulminating [7,10] . Metabolic signs of hypercortisolism are prevalent.…”

Section: Discussionmentioning

confidence: 99%

Ectopic Cushing's Syndrome: Still Much to Understand. A Case Report and a Mini-Review

Pernazza

Somma

Tsalouchos

et al. 2023

SN Compr. Clin. Med.

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Introduction-We describe the case of a patient Ectopic Cushing's syndrome without founding any extrapituitary tumour secreting adrenocorticotropic hormone (ACTH).In the literature there are only a few reported cases and generally the site of the tumor is subsequently identi ed. In addiction we performed a mini-review of the literature.Case report-We report the case of a patient with lower extremity edema, weight gain, uncontrolled hypertension, hypokalemic metabolic alkalosis and hyperglycemia suffering from ACTH-dependent Cushing's syndrome. None of the instrumental examinations allowed us to locate the source of ACTH secretion. Despite a medical therapy with maximum dosage of steroidogenesis inhibitors a bilateral adrenalectomy was performed due to the development of a life-threatening hypercortisolism. After 12 months from surgery follow-up is still negative for ACTH-secreting tumour.Conclusion-In a lot of cases Cushing's syndrome is still nowadays not diagnosed although current advanced techniques. In occult Cushing's syndrome bilateral adrenalectomy is the best therapy when a life-threatening hypercortisolism develops. The ACTH-secreting tumour could be occult for many years after the onset of symptoms. Follow-up in patients with occult ectopic Cushing's syndrome undergoing bilateral adrenalectomy should be prolonged for life

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“…The ECS originate primarily from NETs of the lung, accounting for 50% [ 1 , 2 ]. NETs arising from the mediastinum are extremely rare; they often originate from the thymus gland (10%) or paraganglionic structures (10%) [3] . NETs account for 2%-4% of all anterior mediastinal neoplasms [4] .…”

Section: Introductionmentioning

confidence: 99%