Abstract
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.
Highlights
Fibrolamellar carcinoma (FLC/FL-HCC) is a quite rare variant of hepatocellular carcinoma (HCC) and is intriguing and poorly understood
Riehle et al have recently highlighted some pathways delineating that mechanistic target of rapamycin complex 1 activation is found in FL-HCC and has been found associated with fibroblast growth factor receptor 1 (FGFR1) overexpression supporting the use of FGFR1 inhibitors and FGFR1 overexpression in patients with FL-HCC
Since 1956, from the first description of this tumor by Edmondson, there have been few progresses in research dealing with FL-HCC
Summary
Fibrolamellar carcinoma (FLC/FL-HCC) is a quite rare variant of hepatocellular carcinoma (HCC) and is intriguing and poorly understood. Adolescents and youth are essentially the population affected by this tumor. Both sexes are involved and Caucasians are often the ethnics of this population. C. Sergi (PFIC) or Byler’s disease are often the underlying disorders at pediatric age harboring the risk to develop HCC, all these conditions may show a cirrhotic rearrangement of the liver architecture [2]. FL-HCC is less than one tenth of all HCCs and patients with FL-HCC have similar symptoms to classic HCC (abdominal pain, ascites, hepatomegaly, obstructive jaundice, and systemic manifestations) and FL-HCC is found to be rarely associated with gynecomastia and Budd-Chiari syndrome. Xray shows a central scar, which looks like similar to the scar seen in presence of focal nodular hyperplasia (FNH), is often calcified, which is uncommon with FNH
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