A rare case of metastatic pancreatic hepatoid carcinoma treated with sorafenib.

Background: Hepatoid carcinoma (HC) is a rare histopathological tumor type with prominent features of hepatoid differentiation, and while most of the reported cases are of gastric origin, ten cases of pancreatic HC have been reported to date. The majority of HC cases are metastatic at presentation, mainly to the liver, lymph nodes, and lungs. They are aggressive, invading, and proliferating in the venous and lymphatic systems, with a behavior similar to that of hepatocellular carcinoma. Diagnosis is challenging: alpha-Fetoprotein, the most useful marker, is not always positive.

Methods: We present the first case of metastatic pancreatic HC treated with sorafenib, an oral multikinase inhibitor approved for advanced hepatocellular carcinoma that has antiangiogenic, pro-apoptotic, and raf-kinase inhibitory properties.

Results: The patient, a 37-year-old male, was diagnosed with hepatoid carcinoma of the pancreas that had metastasized to liver, lungs, and lymph nodes. The cytokeratin (CK) profile was useful for the diagnosis: Both the hepatoid and adenocarcinoma components of the tumors were CK18+, CK19+, and CK20+/-, whereas normal and neoplastic hepatocytes are CK18+, CK19-, and CK20-. Amylase, lipase, and liver enzyme levels were elevated, but bilirubin was normal. Treatment with sorafenib resulted in more than 7 months of progression-free survival. Therapy was discontinued after 8 months when his bilirubin level increased dramatically. Signs of liver failure resolved temporarily with insertion of a biliary stent, but his condition deteriorated and he died 3 months later, 1 year after diagnosis.

Conclusion: In the absence of evidence-based experience with this rare and aggressive tumor and given its similarities with hepatocellular carcinoma, sorafenib should be considered as a possible treatment.