Fibrolamellar Carcinoma: A Distinct Variant of Hepatocellular Carcinoma That Is Still Surrounded by Unveils Mysteries

Fibrolamellar carcinoma (FL-HCC) is overall a quite rare variant of hepatocellular carcinoma (HCC) that is intriguing and poorly understood at same time. In humans, it affects essentially the young population (children and young adults) in the peculiar absence of underlying liver diseases. FL-HCC has distinctive clinico-pathologic features with metastases frequently found in the regional lymph nodes, but also peritoneum and lungs. Although initially considered harboring an indolent course, survival outcomes have been recently updated pointing to a neoplasm concealing many aspects of biogenesis. In this review, morphology, molecular pathways, and prognosis of this rare tumor are presented