Introduction: Adrenocortical carcinoma is a rare malignancy with an incidence of 12 per milion population. We present our experience of three patients with adrenal incidentalomas which were subsequently confirmed to be metastatic adrenocortical carcinoma.
Discussion: Our first case presented at 56 years of age with abdominal pain in the background of recent type 2 diabetes and history of alcohol related pancreatitis. CT showed a right adrenal mass more than 100 mm in diameter, multiple lung nodules and a lesion in the liver. He was cushingoid on examination and failed to suppress following overnight dexamethasone with a cortisol of 615 nmol/L. Treatment with metyrapone commenced initially and mitotane introduced one month later. The second case presented at 65 years of age with several month history of polyuruia, polydipsia and worsening fatigue. Initial investigations showed blood glucose of 26.7 mmol/L, potassium 2.7 mmol/L and raised liver function tests. Chest x-ray revealed two opacities in the right lower zone. Subsequent CT showed 111×100×102 mm heterogeneous mass in right adrenal with metastasis to liver, spine and lung. Patient profoundly Cushingoid with severe proximal myopathy and a cortisol of 801 nmol/L following overnight dexamethasone. Metyrapone started with symptomatic improvement and then switched to mitotane monotherapy. Treatment complicated by multifocal pneumonia and patinet died at approximately two months from diagnosis following respiratory arrest. The third case, presented at 45 years of age with right sided back and abdominal pain. CT showed an 86×81 mm mass in right adrenal with multiple small lesions in spleen. No clinical features of hypercortisolism on examination and following two normal 24-hour urine free metanephrine collection, he underwent successful adrenalectomy.
Conclusion: The majority of patients with adrenocortical cancer present with rapidly progressive Cushings syndrome. In those with limited disease, complete tumor removal offers the best chance of cure while mitotane can be used in those with advanced disease.