Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy of the adrenal cortex, with an incidence of 1-2 cases per million per year. Due to its rarity, ACC presents significant diagnostic and therapeutic challenges.
We report a case of a 56-year-old male, ECOG 0, who presented with hyperglycaemia (BSL 26.8 mmol/L) without ketosis, rapid atrial fibrillation, and a urinary tract infection. His diabetes control had deteriorated significantly over the past 12 months with his HbA1c increasing from 6.4% to 11.8%. He had known bilateral adrenal lesions on a 2020 computer tomography (CT) scan for which he had not any further investigation or follow up.
Mild epigastric pain led to a CT abdomen which revealed a 9cm heterogeneous mass in the right adrenal gland with metastases in the liver, lung, and lymph nodes. Hormonal assays indicated a high 24h urinary cortisol (1276 nmol) with a suppressed ACTH (<1.0 pmol/L), elevated dehydroepiandrosterone sulfate (>27.1 µmol/L), and oestradiol (894 pmol/L), consistent with a functional adrenal tumour. Liver biopsy confirmed ACC.
Over his 2-week admission, he deconditioned rapidly with proximal myopathy, his ECOG declining to 3, likely due ongoing cortisol excess . He started chemotherapy with cisplatin, etoposide, and doxorubicin, with plans for mitotane. Cisplatin was changed for carboplatin due to ototoxicity concerns.
Before the addition of mitotane, he suffered large volume melena with haemodynamic instability from a duodenal bleed requiring upper gastroscopy and IR embolisation.
After this, he transitioned to palliative care and died comfortably 6 weeks after his initial presentation. This case report highlights the rapid progression and complexity of managing ACC, highlighting the diagnostic and therapeutic challenges posed by this rare malignancy. Early detection and close monitoring of adrenal lesions, along with timely follow-up, are crucial in diagnosing and managing ACC effectively. Managing cortisol excess is vital to prevent rapid patient deconditioning.