A clinical perspective on ectopic Cushing's syndrome.

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

Journal: Trends in endocrinology and metabolism: TEM 2024;35(4):347-360

PMID: 38143211

Free Full Text on Elsevier Science View on PubMed

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.

Address: 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.; Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden; Department of Pathology and Cancer Diagnostics, Karolinska University Hospital Solna, Stockholm, Sweden.; Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia, Australia; Adelaide Medical School, University of Adelaide, Adelaide, South Australia, Australia.; Department of Endocrinology, Karolinska University, Stockholm, Sweden; Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden. Electronic address: [email protected].

DOI: 10.1016/j.tem.2023.12.003

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MeSH Terms: Humans, Cushing Syndrome, Adrenocorticotropic Hormone, Neuroendocrine Tumors, Pituitary Gland, Adrenal Gland Neoplasms