Fibrolamellar hepatocellular carcinoma:A rare but unpleasant event  

作　　者：Walaa Abdelhamed Mohamed El-Kassas 

机构地区：[1]Department of Endemic Medicine,Sohag University,Sohag 14322,Egypt [2]Department of Endemic Medicine,Faculty of Medicine,Helwan University,Cairo 11795,Egypt

出　　处：《World Journal of Gastrointestinal Oncology》2022年第6期1103-1114,共12页世界胃肠肿瘤学杂志（英文版）（电子版）

摘　　要：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 chemoresponsive than the conventional HCC,however,in advanced cases,multimodality treatments can be effective.Recent advances in molecular studies of FLC have found a unique DNAJB1–PRKACA fusion transcript in most of the cases studied.The review aims to describe clinical characteristics,diagnostic methods,and therapeutic modalities for this rare tumor to raise awareness among clinicians and surgeons.

关 键 词：Fibrolamellar carcinoma Hepatocellular carcinoma HEPATITIS CIRRHOSIS Viral hepatitis infection 

分 类 号：R735.7[医药卫生—肿瘤]