A clinical perspective on ectopic Cushing’s syndrome

Ragnarsson, Oskar; Juhlin, C. Christofer; Torpy, David J.; Falhammar, Henrik

doi:10.1016/j.tem.2023.12.003

Cited by 5 publications

(3 citation statements)

References 74 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Aggressive pituitary adenomas with a large amount of cortisol secretion, rapid onset of catabolic signs of hypercortisolism, and acute severe comorbidities (such as hypertension with hypokalemia, mainly due to 11β-hydroxysteroid dehydrogenase type 2 saturation [54]) mimic a paraneoplastic cortisol excess. On the contrary, some patients with indolent and small, well-differentiated (mainly bronchial) EAS present only with the common signs of cortisol excess [39]. In the context of ACTH-dependent forms, ACTH levels tend to be higher in the case of ectopic secretion compared to those from a pituitary origin; however, there is considerable overlap, so the ACTH measurement alone is not sufficient to distinguish the two conditions [37,54].…”

Section: Differential Diagnosis Of Adrenocorticotropic Hormone-depend...mentioning

confidence: 99%

“…small well differentiated (mainly bronchial) EAS present only with the common signs of cortisol excess [39].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

“…If the BIPSS does not show a significant ACTH gradient between the lower sinus and periphery, a total-body computed tomography (CT) is recommended in the suspicion of an EAS. Although the thorax is the most likely site of ACTH-secreting tumors [37][38][39], the identification of these lesions is often difficult and they are detected only in 65 % of cases during the first assessment. Nuclear medicine improves the sensitivity of conventional radiology when the NET is not identified; however, its high diagnostic accuracy (75 % of patients with initial occult EAS with conventional imaging) is not confirmed in large series.…”

Section: Imagingmentioning

confidence: 99%

See 2 more Smart Citations

Subtyping of Cushing’s Syndrome: A Step Ahead

Tizianel,

Barbot,

Ceccato

2024

Exp Clin Endocrinol Diabetes

View full text Add to dashboard Cite

Cushing’s Syndrome (CS) is a rare disease due to chronic endogenous cortisol secretion. In the last years, new acquisitions enlarged the spectrum of differential diagnosis, historically divided into ACTH-dependent and ACTH-independent forms. Moreover, the increased awareness of the detrimental cortisol effects on cardiometabolic health and the risk of cardiovascular events lead to increased diagnosis of mild forms, especially in the context of adrenal incidentalomas. We provide and up-to-date narrative review of the most recent literature regarding the challenges of CS diagnosis. After the description of the diagnostic tools available, we describe the characterization of functional non-neoplastic hypercortisolism (formerly known as pseudo-Cushing state), then we report the subtyping of the different conditions of hypercortisolism: the differential diagnosis of ACTH-dependent forms and the management of adrenal hypercortisolism, with peculiar attention to the new genetic classification of adrenal CS, mild autonomous cortisol secretion and bilateral adrenal adenomas.

show abstract

Section: Differential Diagnosis Of Adrenocorticotropic Hormone-depend...mentioning

confidence: 99%

“…small well differentiated (mainly bronchial) EAS present only with the common signs of cortisol excess [39].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Section: Imagingmentioning

confidence: 99%

See 1 more Smart Citation

Subtyping of Cushing’s Syndrome: A Step Ahead

Tizianel,

Barbot,

Ceccato

2024

Exp Clin Endocrinol Diabetes

View full text Add to dashboard Cite

show abstract

Adrenal tumors in patients with neuroendocrine neoplasms

Falhammar,

Stenman,

Juhlin

et al. 2024

Endocrine

View full text Add to dashboard Cite

Purpose To study the prevalence of primary adrenal tumors and adrenal metastases in patients with neuroendocrine neoplasms (NENs) and describe these in detail. NENs can be further divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC). Methods A review of medical files was conducted for all patients who underwent a 68Gallium-DOTATOC-PET/CT during 2010−2023 or adrenalectomy during 1999-2023 at the Karolinska University Hospital. Results In total, 68Gallium-DOTATOC-PET/CT was performed on 1750 individuals with NEN, among whom 12 (0.69%) had adrenal tumors. Of these, 9 (0.51%) were NEN metastases. Out of 1072 adrenalectomies, 4 (0.37%) showed evidence of NEN metastases. Thus, 16 patients with NEN exhibited adrenal tumors. The adrenal tumors were found on average 5 years after the NEN diagnosis and 19% of the adrenal tumors with simultaneous NEN were benign. Few had all adrenal hormones measured. None had an adrenal insufficiency nor an adrenal biopsy. Another synchronous metastasis was found in 69% at the time of the adrenal tumor discovery. During the median 2-year follow-up, 38% of the subjects had deceased (with the exclusion of individuals presenting supposedly benign adrenal tumors 31%) all due to tumor complications. A comparison between individuals identified through 68Gallium-DOTATOC-PET/CT and those who underwent adrenalectomy revealed a higher prevalence of NETs in the former group and NECs in the latter group. Conclusion Adrenal primary tumors and adrenal metastases are infrequent occurrences in patients with NEN. Most cases involved the presence of NEN metastasis upon the initial discovery of adrenal tumors. The overall prognosis was found to be favorable.

show abstract