Hepatoid adenocarcinoma (HAC) is a rare tumor with produces an alpha-fetoprotein (AFP) mimics Hepatocellular Carcinoma (HCC). Adrenal HAC is exceeding rare. Here we report the extremely high alpha-fetoprotein (AFP) producing adrenal HAC, the first case report of Thailand.
A 47-year-old male presented with severe left flank pain and progressive dyspnea for 2 months. He also lost his bodyweight for 15 kg within 2 months. Physical examination showed a huge mass at the left flank area by bimanual palpation. CT scan of the whole abdomen revealed a 5.5 x 7 cm enhancing mass involving the left adrenal gland and left hemidiaphragm, a 3.7 x 3.3 cm early enhancing mass at liver segment VII on arterial phase and rapid washout on porto-venous phase, and multiple hypodense masses in both lobes of the liver. AFP level was 321,495 ng/mL. A liver biopsy was done. This patient was diagnosed with advanced adrenal hepatoid adenocarcinoma due to the immunohistochemistry studies (IHC) positive for AE1/AE3, CAM5.2, Arginase-1, and Glypican-3 but negative for CK7, CK20, CK19, Inhibin A, Chromogranin A, Synaptophysin, S100, and HepPar-1. Unfortunately, the tumor was progression and involving through the stomach. He developed massive upper-GI bleeding and passed away 3 weeks after diagnosis.
Adrenal HAC is an extremely rare cancer. It is difficult to diagnosis which HCC, paraganglioma, and pheochromocytoma should be excluded. Surgical resection is preferred if resectable condition. However, there is no standard treatment for systemic therapy.
Acknowledge to Phramongkutklao Hospital (PMK) Tumour Board, Medical Oncology division, Endocrinology division, and Clinical Nutrition division, Department of Internal medicine, Department of Pathology, and Department of Radiology for collaborating to diagnose and treat this patient