Endocrine Abstracts

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. They are believed to arise from cortical fragments arrested during embryologic migration and have been found close to the adrenal gland or along the path of gonadal descent. 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. 1 mg overnight dexamethasone suppressed 0900 h cortisol was 548 nmol/l [<50 nmol/l] with an undetectable ACTH <3.0 pg/ml (7.2 – 63.3 pg/ml). DHEAS 5.3 μmol/l (0.5–5.6μmol/l) and androstenedione 3.49 nmol/l (1.39 – 9.77 nmol/l) were normal. Testosterone was suppressed <0.4 nmol/l (0.1–1.4 nmol/l). Imaging revealed a 6×6×4.5 cm right-sided presumed adrenal lesion with Hounsfield units >20, a pancreatic lesion [2.5×1.6 cm], and bilateral pulmonary nodules [0.9×0.8 cm, 0.7×0.6 cm, 0.3 cm]. Right adrenalectomy was performed and histology was consistent with an extra-adrenal ACC [Weiss score 5/9] within the peri-adrenal adipose tissue. The resected adrenal gland was normal. Lung biopsy confirmed metastatic ACC tissue and endoscopic ultrasound-guided biopsy of the pancreatic lesion revealed a pancreatic neuroendocrine tumour which was diagnosed biochemically to be an insulinoma. Genetic assessment confirmed MEN-1. Adrenal lesions in MEN-1 syndrome have significant malignant potential. ACCs are reported in 2.5–6% of MEN-1 patients. This case highlights the importance of screening for MEN-1 in at-risk patients and the need for close clinical follow-up. To our knowledge, this is the first case report of extra-adrenal ACC in MEN-1 syndrome.