Fibrolamellar hepatocellular carcinoma

Area of Science:

• Hepatobiliary Pathophysiology
• Oncology
• Radiology

Background:

• Fibrolamellar hepatocellular carcinoma (HCC) is increasingly recognized as a distinct clinicopathologic entity.
• It presents with a better prognosis compared to conventional HCC, particularly in patients without cirrhosis.
• This subtype often affects younger individuals with normal tumor marker levels and no traditional HCC risk factors.

Purpose of the Study:

• To delineate the clinicopathologic, histologic, and radiologic features of fibrolamellar HCC.
• To evaluate the potential for pre-biopsy diagnosis using a combination of clinical and imaging data.

Main Methods:

• Retrospective analysis of 17 patients diagnosed with fibrolamellar HCC.
• Review of clinical data, laboratory results (including alpha fetoprotein levels), and histopathologic findings.
• Correlation of imaging findings from plain films, sonography, and computed tomography (CT).

Main Results:

• The mean age of patients was 20 years, with normal alpha fetoprotein levels and absence of typical HCC risk factors.
• Histologic examination revealed deeply eosinophilic polygonal hepatocytes and abundant fibrous stroma.
• Radiologic findings included calcification on plain films (5/13) and CT (4/10), and a characteristic central scar (hypodense on CT) in two cases. Sonography typically showed a homogeneous, echogenic mass.

Conclusions:

• Fibrolamellar HCC is a unique subtype characterized by specific demographic, clinical, and histologic features.
• Radiologic findings, particularly calcification and a central scar, along with clinical presentation, can suggest the diagnosis prior to biopsy.
• Integrated diagnostic approaches improve the likelihood of pre-procedural diagnosis for fibrolamellar HCC.