Fibrolamellar Carcinoma: A Concise Review

Area of Science:

• Hepatobiliary pathology
• Oncology
• Gastroenterology

Background:

• Fibrolamellar carcinoma (FLC) is a rare primary liver cancer.
• It predominantly affects young individuals with non-cirrhotic livers.
• Patients typically present with a large solitary liver mass and vague symptoms.

Purpose of the Study:

• To review the clinical features, pathology, molecular genetics, diagnosis, treatment, and prognosis of FLC.
• To highlight key diagnostic markers and differential diagnoses.
• To discuss current and potential therapeutic strategies.

Main Methods:

• Review of clinical, histological, and molecular data.
• Analysis of immunohistochemical markers for differential diagnosis.
• Correlation of prognosis with tumor resectability and treatment options.

Main Results:

• FLC is characterized by specific histological features and protein kinase A activation.
• Immunohistochemistry (cytokeratin 7, CD68, HepPar-1) aids in distinguishing FLC from hepatocellular carcinoma and cholangiocarcinoma.
• Tumor resectability is a strong prognostic factor.

Conclusions:

• FLC diagnosis requires a combination of clinical, histological, and immunohistochemical findings.
• Prognosis is comparable to classical hepatocellular carcinoma in non-cirrhotic livers.
• Treatment strategies include surgery, liver transplant, chemotherapy, and embolization.