Join the RareGuru Community
To connect, share, empower and heal today.
Don’t fight Fibrolamellar carcinoma alone.
Find your community on the free RareGuru App.Fibrolamellar carcinoma (FLC) is a rare form of liver cancer which is generally diagnosed in adolescents and young adults (before age 40). Many people with early FLC have no signs or symptoms of the condition. When present, symptoms are often nonspecific (i.e. abdominal pain, weight loss, malaise) and blamed on other, more common conditions. The exact underlying cause of FLC is poorly understood. Unlike other forms of liver cancer, FLC typically occurs in the absence of underlying liver inflammation or scarring; thus, specific risk factors for this condition remain unidentified. FLC is typically treated with surgical resection.
Source: GARD Last updated on 05-01-20
Many people with early fibrolamellar carcinoma (FLC) have no signs or symptoms of the condition. When present, symptoms are often nonspecific and blamed on other, more common conditions. Signs and symptoms may include:
Other signs and symptoms that have been reported less commonly include:
Last updated on 05-01-20
The exact underlying cause of fibrolamellar carcinoma (FLC) is poorly understood. Other forms of liver cancer are often associated with liver cirrhosis (scarring of the liver) which may be caused by alcohol abuse; autoimmune diseases of the liver; Hepatitis B or C viral infections; chronic inflammation of the liver; and/or hemochromatosis. However, FLC typically occurs in the absence of underlying liver inflammation or scarring; thus, specific risk factors for this condition remain unidentified.
Recent research suggests that a deletion on chromosome 19 may play a key role in the formation of FLC. This deletion is called a "somatic mutation" since it is only present in the cells of the liver. Somatic mutations accumulate during a person's lifetime and are not inherited or passed on to future generations.
Last updated on 05-01-20
If fibrolamellar carcinoma (FLC) is suspected based on the presence of certain signs and symptoms, imaging studies such as ultrasound, MRI scan and/or CT scan are typically recommended for diagnosis and staging. Unlike other forms of liver cancer, serum alpha fetoprotein is typically not elevated in FLC.
Medscape Reference's Web site offers more specific information on the diagnosis of FLC. Please click on the link to access this resource.
Last updated on 05-01-20
The long-term outlook (prognosis) for people with fibrolamellar carcinoma (FLC) is generally better than other forms of liver cancer, particularly when treated with surgical resection. In fact, reported 5-year survival rates range from 51 to 70% following liver resection.
Unfortunately, the prognosis is less favorable in people with unresectable disease. The median survival of these patients is approximately 14 months.
Last updated on 05-01-20
The standard treatment for fibrolamellar carcinoma (FLC) is surgical resection. Due to the rarity of the condition, there is limited information to support the use of other treatment options and there is no standard chemotherapy regimen. However, other treatments may be considered if surgical resection isn't an option. For example, liver transplantation may be considered in patients who are not candidates for partial resection (removing a portion of the liver).
Medscape Reference's Web site offers more specific information on the treatment and management of FLC. Please click the link to access this resource.
Last updated on 05-01-20
The Fibrolamellar Tissue Repository is a tissue bank for fibrolamellar samples obtained during surgery. This repository is a resource for Rockefeller University and other approved academic institutions who are interested in performing scientific research that will improve the diagnosis, treatment, and understanding of this rare disease.
Last updated on 04-27-20
Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!
To connect, share, empower and heal today.