Hepatoid adenocarcinoma masquerading as adrenocortical carcinoma in an elderly male

Background: Hepatoid adenocarcinoma is a malignant lesion arising from an anatomic site other than the liver with histological features similar to hepatocellular carcinoma. Hepatoid adenocarcinoma has been reported from various anatomical sites, but adrenal hepatoid adenocarcinomas are extremely rare.Case Description: A 70-year-old male with well-controlled hypertension presented with chronic abdominal pain and was found to have a large left-sided supra renal mass. The lesion showed radiological features suggestive of malignancy with local invasion. There was biochemical evidence of primary aldosteronism and a non-suppressed overnight dexamethasone suppression test. He underwent open left-sided adrenalectomy: histologically the tumour demonstrated features typical of a hepatoid carcinoma with bile production and immunohistochemical staining for Hep Par-1 and CD10 demonstrating a hepatic canalicular pattern. Adrenocortical-specific immunohistochemical markers (Inhibin and melan A) and neuroendocrine markers (synaptophysin and chromogranin) were negative. He did not have clinical or biochemical evidence of cirrhosis, Hepatitis B or C infection. Triple-phase CT scanning of the abdomen before resection of the adrenal lesion and five months following surgery did not show any significant lesion in the liver suggestive of primary hepatocellular carcinoma, except an 8 mm non enhancing benign-appearing cystic lesion.Conclusion: Most hepatoid adenocarcinomas originate from the gastro-intestinal tract. We present a patient with hepatoid adenocarcinoma masquerading as adrenocortical carcinoma due to misleading imaging and biochemical evidence. Very little is known about their pathogenesis, especially those with an atypical site of origin such as the adrenal gland.