Fibrolamellar hepatocellular carcinoma with intrapancreatic nodal metastases masquerading as pancreatic neuroendocrine tumour with hepatic metastases

Fibrolamellar hepatocellular carcinoma (FL-HCC) is a primary liver tumour presenting at a young age. Aggressive surgery of FL-HCC is the mainstay of management unlike other malignancies where metastatic stage precludes curative surgery. There are limited reports of response of FL-HCC to systemic therapies predominantly owing to its rarity. In the index case, a young boy presented with the awareness of abdominal lump with minimal systemic complaints. Radiological imaging showed arterially enhancing liver and pancreatic lesions leading to clinical suspicion of non-functional pancreatic neuroendocrine tumour with neuroendocrine liver metastases in non-cirrhotic liver. Histopathological examination with immunohistochemistry following left lateral hepatectomy with pancreaticoduodenectomy and lymphadenectomy showed FL-HCC with lymph nodal metastases involving intrapancreatic lymph node and station 7, 8 lymph nodes. The patient had an uneventful postsurgical recovery and was kept under close follow-up. FL-HCC with nodal metastasis involving intrapancreatic lymph node is an unusual phenomenon and a challenging clinicopathological diagnosis.