Hepatoid carcinoma of pancreas: Diagnostic challenges and literature review

Hepatoid carcinomas are a set of extrahepatic neoplasms representing focal hepatocellular carcinoma on morphology and immunohistochemistry. Hepatoid carcinoma is reported primarily in the ovary and stomach, but its presence in the urinary tract, lung, and biliary tract has also been reported. Clinical presentation, diagnosis, treatment, and prognosis of pancreatic hepatoid carcinomas are not well understood due to the rarity of available literature. Our case was a female of 33 years of age who had pain in her abdomen for three months. Serum carcinoembryonic antigen was raised, and alpha-fetoprotein was normal at baseline. A contrast-enhanced computed tomography scan of the abdomen, pelvis, and thorax revealed a pancreatic body mass with regional nodal metastases. Whole-body positron emission tomography/computed tomography scan showed the mass in the body of the pancreas encasing the splenic vein and splenic-portal confluence with regional nodal involvement. Biopsy and immunohistochemistry of the pancreatic mass revealed pancreatic hepatoid carcinoma. Post-discussion in multidisciplinary tumor board, it was found to be unresectable and hence started on modified FOLFIRINOX for four cycles, following which the patient developed multiple liver lesions, eastern cooperative oncology group performance status deteriorated to IV, following which the patient kept on best supportive care and died after four months. Due to the rarity of this tumor and as per the limited literature available, the mainstay treatment for pancreatic hepatoid carcinoma remains complete surgical resection with significantly improved survival. The prognosis cannot be predicted precisely due to a lack of evidence-based data.