Plain English summary of "Plain English Summary of "Advances in Fibrolamellar Hepatocellular Carcinoma: a Review"
Published December 2014 in European Journal of Pediatric Surgery
Fibrolamellar hepatocellular carcinoma (FLHCC) accounts for less than 1% of all liver cancer cases that originate in the liver. Surgery to remove the entire tumor remains the cornerstone of treatment of FLHCC; however, this may not always be possible as some patients present with advanced stage disease with larger tumors. As a rare liver cancer, FLHCC currently does not have a standardized chemotherapy regimen as other cancers do (i.e. breast cancer). Some investigators have looked at various chemotherapy regimens with varied results including: gemcitaibine/oxaliplatin (GEMOX) or 5-flurouracil (5-FU) with interferon alpha (IFN-a) and Folinic acid 5-FU/Oxaliplatin (FOLFOX). Various scientists have been investigating the biology of the tumor. Others have studied the mammalian target of rapamycin complex 1 (mTORC1) and fibroblast growth factor receptor 1 (FGFR1) signaling in FLHCC tumors implicating a potential role of these pathways in the disease process.
Advances in whole genome sequencing have revealed what may be the closest explanation for why FLHCC develops a chimera between two genes (DNAJB1-PRKACA) that was originally found in 15 out of 15 tumor samples.
FLHCC is associated with more favorable outcomes compared with conventional hepatocellular carcinoma (HCC) when treated with surgery, with 5-year survival rates ranging from 58-82%.
Multiple studies have shown the presence of FLHCC outside the liver is a predictor of overall and recurrence free survival.
Research in improved methods of diagnosis and response to non-operative therapies are still needed.
--- Dr. Benjamin Farber
Fibrolamellar Registry Scientific Advisory Board