Published online Jun 15, 2022. doi: 10.4251/wjgo.v14.i6.1103
Peer-review started: January 15, 2022
First decision: March 13, 2022
Revised: March 19, 2022
Accepted: May 8, 2022
Article in press: May 8, 2022
Published online: June 15, 2022
Processing time: 145 Days and 3.4 Hours
Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), comprising 1%–9% of all HCCs. FLC is a poorly understood malignancy, which seems to be more prevalent in young patients with no underlying liver diseases. The term “fibrolamellar” is derived from thick fibrous collagen bands surrounding the tumor cells. Unlike HCC, cirrhosis and viral hepatitis infection are not predisposing to FLC, and it is not associated with elevations in serum alpha-fetoprotein. FLC patients often present with vague abdominal pain, nausea, malaise, and weight loss. Most cases present are at an advanced stage at the time of initial diagnosis. However, curative treatment options can still be offered to up to 70% of patients. Surgery (resection/liver transplantation) is the mainstay of treatment and the only potentially curative option. FLCs have been less chemo-responsive than the conventional HCC, however, in advanced cases, multi
Core Tip: Fibrolamellar carcinoma (FLC) is a rare liver cancer that displays unique features in behavior and clinical findings from conventional hepatocellular carcinoma (HCC). No certain underlying trigger is detected in FLC. Alpha-fetoprotein levels are normal, unlike in traditional HCC. Surgery (resection/liver transplantation) is the current mainstay of treatment and remains the only curative option. FLCs have been less chemo-responsive than the conventional HCC. Controlled trials evaluating checkpoint inhibitors in FLC are lacking. In this review, we collect and summarize current evidence and clinical experience of conversion therapy, highlight remaining problems and challenges for further research.