Abstract
Hepatoid adenocarcinoma is a rare extrahepatic malignancy with histological features and biochemical profile similar to hepatocellular carcinoma. A 38-year-old male with a 10-year history of ulcerative colitis on treatment was found to have a large rectal mass with locoregional lymphadenopathy and distant spread to peri-portal nodes, hepatic parenchyma, and portal vein. Based on elevated serum AFP levels and immunohistochemical staining of rectal tissue, he was diagnosed with metastatic hepatoid adenocarcinoma and was treated with ten cycles of palliative folinic acid, fluorouracil and oxaliplatin (FOLFOX). Following an excellent response to FOLFOX, he was treated with curative intent with 5-fluorouracil (5-FU)-based neoadjuvant chemoradiation followed by proctocolectomy for local cancer control and ulcerative colitis treatment. This was followed by stereotactic body radiation therapy (SBRT) to the liver. The patient had no evidence of relapse three months after his last day of SBRT. This is the first report of comprehensive genomic testing in a patient with hepatoid adenocarcinoma. Genomic testing revealed a TP53 mutation with wild-type K-RAS. The tumor also revealed low (5-10%) expression of programmed death-ligand 1 (PD-L1) and stable microsatellite instability. Though the patient had metastatic disease, he was treated with curative intent with excellent response. This case highlights the fact that aggressive management might lead to good response in this highly aggressive malignancy.
Published Version
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