Fibrolamellar hepatocellular carcinoma

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Fibrolamellar hepatocellular carcinoma
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Abdominal pain, weight loss, jaundice, nausea
Complications	Metastasis, liver failure
Onset	Typically in adolescents and young adults
Duration	Chronic
Types	Primary liver cancer
Causes	Unknown
Risks	No known risk factors
Diagnosis	Imaging studies, biopsy
Differential diagnosis	Hepatocellular carcinoma, cholangiocarcinoma
Prevention	None
Treatment	Surgical resection, chemotherapy, liver transplantation
Medication	Chemotherapy drugs
Prognosis	Variable, generally better than conventional hepatocellular carcinoma
Frequency	Rare
Deaths	N/A

Fibrolamellar hepatocellular carcinoma - High magnification

Fibrolamellar Hepatocellular Carcinoma (FL-HCC) is a rare form of liver cancer that typically affects adolescents and young adults who do not have a history of liver disease.

Epidemiology

FL-HCC accounts for a small percentage of all HCC cases. It is more common in young adults and has no significant gender predilection. Unlike most forms of HCC, FL-HCC is not associated with chronic liver disease or cirrhosis.

Pathophysiology

The pathophysiology of FL-HCC is not fully understood. It is known that the tumor cells in FL-HCC are characterized by large, pale eosinophilic cytoplasm and prominent nucleoli. The tumor cells are arranged in cords that are separated by fibrous bands, giving the tumor its characteristic "fibrolamellar" appearance.

Clinical Presentation

Patients with FL-HCC often present with nonspecific symptoms such as weight loss, abdominal pain, and a palpable mass in the abdomen. Some patients may also present with jaundice.

Diagnosis

The diagnosis of FL-HCC is typically made based on imaging studies such as CT or MRI, and confirmed by liver biopsy. The tumor often appears as a large, solitary mass with a central scar on imaging studies.

Treatment

The mainstay of treatment for FL-HCC is surgical resection, which can be curative if the tumor is localized. For patients with advanced disease, chemotherapy and targeted therapies may be used.

Prognosis

The prognosis of FL-HCC is generally better than that of other forms of HCC, with a 5-year survival rate of approximately 50-70%. However, the prognosis can vary widely depending on the stage of the disease at diagnosis and the patient's overall health.

See Also

• Hepatocellular carcinoma
• Liver cancer
• Computed tomography
• Magnetic resonance imaging

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