title: Frequency of Cushing's syndrome due to ACTH-secreting adrenal medullary lesions: a retrospective study over 10 years from a single center.
abstract: Cushing's syndrome due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions has occasionally been described. We retrospectively reviewed all 164 cases of Cushing's syndrome and 77 cases of pheochromocytomas during 10 years. Of all cases with Cushing's syndrome, only two cases (1.2 %) were due to ectopic adrenocorticotropic hormone production from adrenal medullary lesions (one case of pheochromocytoma and one case of adrenal medullary hyperplasia). Of all pheochromocytomas only the above-mentioned case (1.3 %) also gave rise to an ectopic adrenocorticotropic hormone syndrome. The clinical presentation of adrenocorticotropic hormone-secreting pheochromocytoma and adrenal medullary hyperplasia can be anything from mild to dramatic. These are rare conditions important to bear in mind in the workup of a patient with Cushing's syndrome or with pheochromocytoma. The identification of ectopic adrenocorticotropic hormone secretion from adrenal medullary lesions can be life-saving.
authors.affiliation: Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden. henrik.falhammar@ki.se.|Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden. henrik.falhammar@ki.se.
authors.forename: Henrik
authors.identifier: http://orcid.org/0000-0002-5622-6987
authors.initials: H
authors.lastname: Falhammar
authors.affiliation: Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.|Department of Clinical Science and Education, Karolinska Institutet, Södersjukhuset, Stockholm, Sweden.
authors.forename: Jan
authors.initials: J
authors.lastname: Calissendorff
authors.affiliation: Department of Endocrinology, Metabolism and Diabetes, Karolinska University Hospital, Stockholm, Sweden.|Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Höybye
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000378:metabolism; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D003480:Cushing Syndrome / Q000453:epidemiology* / Q000209:etiology; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D010673:Pheochromocytoma / Q000150:complications* / Q000378:metabolism; D012189:Retrospective Studies
chemical_list: D000324:Adrenocorticotropic Hormone
keywords: Adrenal medullary hyperplasia; Cushing; Ectopic; Pheochromocytoma