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Endocrine Abstracts (2025) 113 WH4.1 | DOI: 10.1530/endoabs.113.WH4.1

1University Hospital Bristol and Weston, Bristol, United Kingdom; 2North Bristol Trust, Bristol, United Kingdom


Adrenocortical carcinoma is a rare and aggressive malignancy, with an incidence of 1–2 cases per million annually. Approximately 50% present with advanced disease, and 60% of cases are hormonally active—most commonly with cortisol excess. This case describes a 63-year-old male farmer who initially presented with chest pain and elevated troponin levels, and was treated for NSTEMI. Further investigation revealed bilateral lung nodules and a right adrenal mass with inferior vena cava (IVC) thrombus. Clinical features included significant weight gain, cushingoid appearance, uncontrolled hypertension on four agents, and hyperglycaemia. Biochemical assessment showed severe hypercortisolaemia with suppressed ACTH, negative plasma metanephrines, and a urine steroid profile suggestive of cortisol excess only. Imaging confirmed a large right adrenal mass invading the adrenal vein and IVC, with indeterminate liver lesions and pulmonary metastases. Metyrapone was initiated for cortisol control and anticoagulation commenced for the IVC thrombus. The case was referred to a specialist Neuroendocrine Tumour (NET) multidisciplinary team (MDT), which recommended debulking surgery following adequate cortisol suppression. Peri-operative management was high-risk, given the background of recent NSTEMI and severe hypercortisolaemia. Metyrapone was stopped preoperatively; hydrocortisone was administered peri-operatively alongside steroid-sparing measures and PCP prophylaxis. The patient underwent open right adrenalectomy, with complete resection of the adrenal mass. Liver nodules were left in situ. Post-operatively, the patient showed significant clinical improvement, including weight loss, better glycaemic and blood pressure control, and improved functional status. Histopathology confirmed adrenocortical carcinoma with lymphovascular invasion, a high Ki-67 index (30–40%), and R2 resection margins. Final staging was ENSAT Stage IV (pT4 pNX pMX). This case highlights the complexity of managing advanced ACC, especially in the context of life-threatening hypercortisolaemia. Debulking surgery, although not curative in metastatic ACC, may offer meaningful symptom relief, improved quality of life, and facilitate subsequent oncological treatment. Early identification and control of cortisol excess are crucial to reducing surgical risk

Volume 113

Society for Endocrinology Clinical Update 2025

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