A clinical perspective on ectopic Cushing's syndrome

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

doi:10.1016/j.tem.2023.12.003

A clinical perspective on ectopic Cushing's syndrome

Trends Endocrinol Metab. 2024 Apr;35(4):347-360. doi: 10.1016/j.tem.2023.12.003. Epub 2023 Dec 23.

Authors

Oskar Ragnarsson¹, C Christofer Juhlin², David J Torpy³, Henrik Falhammar⁴

Affiliations

¹ Department of Internal Medicine and Clinical Nutrition, Institute of Medicine at Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden; Wallenberg Center for Molecular and Translational Medicine, University of Gothenburg, SE-413 90 Gothenburg, Sweden.
² Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Cancer Diagnostics, Karolinska University Hospital Solna, Stockholm, Sweden.
³ Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia, Australia; Adelaide Medical School, University of Adelaide, Adelaide, South Australia, Australia.
⁴ Department of Endocrinology, Karolinska University, Stockholm, Sweden; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden. Electronic address: henrik.falhammar@ki.se.

PMID: 38143211
DOI: 10.1016/j.tem.2023.12.003

Abstract

Cushing's syndrome (CS) refers to the clinical features of prolonged pathological glucocorticoid excess. About 10-20% of individuals with CS have ectopic CS (ECS), that is, an adrenocorticotropin (ACTH)-producing tumour outside the pituitary gland. ACTH-secreting neuroendocrine neoplasia (NENs) can arise from many organs, although bronchial NEN, small cell lung cancer (SCLC), pancreatic NEN, thymic NEN, medullary thyroid cancer (MTC), and pheochromocytoma are the most common. Patients with ECS frequently present with severe hypercortisolism. The risk of life-threatening complications is high in severe cases, unless the hypercortisolism is effectively treated. A good outcome in ECS requires a methodical approach, incorporating prompt diagnosis, tumour localization, control of cortisol excess, and resection of the primary tumour when possible.

Keywords: ectopic ACTH; ectopic CRH; hypercortisolism; imaging; outcomes; treatment.

Publication types

Review

MeSH terms

Adrenal Gland Neoplasms*
Adrenocorticotropic Hormone
Cushing Syndrome*
Humans
Neuroendocrine Tumors*
Pituitary Gland / pathology

Substances

Adrenocorticotropic Hormone