True regarding fibrolamellar carcinoma (FLC) of liver are all the following EXCEPT
Ans. d (Occurs in preexisting cirrhotic liver) (Ref. Harrison 18th/Ch. 92).Fibrolamellar carcinoma differs from typical HCC in that it tends to occur in young adults without underlying cirrhosis. It is non- encapsulated. Longer survival if treated.Fibrolamellar carcinoma is typically a disease of younger adults, often teenagers and predominantly females. It is AFP-negative, but patients typically have elevated blood neurotensin levels, normal liver function tests, and no cirrhosis. Radiology is similar for HCC, except that characteristic adult-type portal vein invasion is less common. Although it is often multifocal in the liver, and therefore not resectable, metastases are common, especially to lungs and locoregional lymph nodes, but survival is often much better than with adult-type HCC. Resectable tumors are associated with 5-year survival >50%. Patients often present with a huge liver or unexplained weight loss, fever, or elevated liver function tests on routine evaluations. These huge masses suggest quite slow growth for many tumors. Surgical resection is the best management option, even for metastases, as these tumors respond much less well to chemotherapy than adult-type HCC. Although several series of OLTX for FL-HCC have been reported, the patients seem to die from tumor recurrences, with a 2- to 5-year lag compared with OLTX for adult-type HCC. Anecdotal responses to gemcitabine plus cisplatin-TACE are reported.HepatoblastomaAngiosarcomaEpithelioid hemangioendothelioma# It is tumor of infancy that typically is associated with very high serum AFP levels.# The lesions are usually solitary, may be resectable, and# Have a better 5-year survival than that of HCC.# It consists of vascular spaces lined by malignant endothelial cells.# Etiologic factors include prior exposure to thorium dioxide, polyvinyl chloride, arsenic, and androgenic anabolic steroids.# Prognosis is not good.# It is of borderline malignancy.# Most cases are benign, but bone and lung metastases occur.# Occurs in early adulthood,# Presents with right upper quadrant pain, and# Is heterogeneous on sonography, hypodense on CT, and without neovascularity on angiography.# Immunohistochemical staining reveals expression of factor VIII antigen.# In the absence of extrahepatic metastases, these lesions can be treated by surgical resection or liver transplantation.
Age group of occurrence is between 20-40 years
Extensive fibrosis
Favorable prognosis
Occurs in pre-existing cirrhotic liver
Liver & Biliary Tract