A Distinct Entity
FLC is not a typical liver cancer. It arises in young individuals with healthy livers, unlike HCC which is linked to cirrhosis or hepatitis.
Estimated U.S. Incidence
~1 in 5 Million
Who Gets FLC? The Patient Profile
FLC has a unique demographic footprint that sets it apart from other liver cancers. It does not discriminate by gender and strikes at a much younger age, creating a distinct patient population.
Median Age at Diagnosis
Peaks in adolescence and young adulthood, but can occur at any age.
Gender Distribution
Affects males and females at a nearly equal rate.
The Genetic Hallmark
The vast majority of FLC tumors are driven by a specific genetic mutation, making it a key focus for diagnosis and future treatments.
Chromosome 19
Deletion Occurs
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DNAJB1-PRKACA
Fusion Gene Created
This chimeric protein drives tumor growth and is a definitive marker for FLC.
Signs & Symptoms
Symptoms are often vague and may only appear as the tumor grows. Early stages are frequently asymptomatic.
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Abdominal Pain
Most common symptom
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Weight Loss
Unexplained
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Fatigue & Malaise
General unwellness
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Nausea
Feeling of sickness
The Path to Diagnosis
Diagnosing FLC requires a multi-step approach, moving from initial suspicion to definitive confirmation through advanced testing.
Clinical Suspicion
Liver mass found in a young patient with no prior liver disease.
Imaging
CT and MRI scans reveal a large, defined mass, often with a central scar.
Biopsy
Histology shows large cells separated by fibrous bands (lamellae).
Definitive Diagnosis
Molecular testing confirms the DNAJB1-PRKACA fusion gene.
Treatment & Survival
Outcomes for FLC are starkly divided by the ability to perform surgery. This remains the only curative option, as the cancer shows poor response to traditional chemotherapy.
5-Year Survival Rate Comparison
Complete surgical removal of the tumor is the single most important factor for long-term survival.
Response to Chemotherapy
FLC is largely resistant to systemic chemotherapy, driving research into targeted and immunotherapies.
FLC vs. HCC: A Tale of Two Cancers
| Feature | Fibrolamellar Carcinoma (FLC) | Hepatocellular Carcinoma (HCC) |
|---|---|---|
| Typical Age | Adolescents & Young Adults (<40) | Older Adults (>50) |
| Underlying Liver | Healthy / Non-cirrhotic | Cirrhosis / Hepatitis |
| Gender | Equal Male/Female Ratio | Male Predominance |
| AFP Tumor Marker | Usually Normal | Often Elevated |
| Key Genetic Driver | DNAJB1-PRKACA Fusion | Various mutations (e.g., TERT, TP53) |