Extra-adrenal adrenocortical cancer associated with multiple endocrine neoplasia type 1

Autor: Deirdre Green, Kate Richards, Brendan Doyle, Chris Thompson, Arnold Hill, Michael W O’Reilly, Mark Sherlock
Jazyk: angličtina
Rok vydání: 2024
Předmět:
Zdroj: Endocrinology, Diabetes & Metabolism Case Reports, Vol 1, Iss 1, Pp 1-5 (2024)
Druh dokumentu: article
ISSN: 2052-0573
DOI: 10.1530/EDM-23-0068
Popis: Adrenocortical carcinoma (ACC) is a rare malignant tumour arising from the adrenal cortex, with an estimated annual incidence of one to two patients per million. Ectopic ACCs are extremely rare. The majority of ACCs are sporadic; however, ACC has been linked with genetic disease processes, including multiple endocrine neoplasia type-1 (MEN-1). We present the case of a 66-year-old lady referred with newly diagnosed diabetes on a background of primary hyperparathyroidism. Examination revealed Cushingoid features, and hormonal evaluation confirmed ACTH-independent Cushing’s syndrome. Morning cortisol after a 1 mg overnight dexamethasone suppression test was 548 nmol/L with an undetectable ACTH
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