Fibrolamellar (fibro-la-mel-lar) Hepatocellular Carcinoma is a rare of liver cancer that usually occurs in young adults who have no history of liver disease. Fibrolamellar has an incidence rate of 1 in 5,000,000 in the population at large. Fibrolamellar is considered an ultra-rare cancer falling well below the definition of ultra-rare, which is fewer than 1000 cases per year. Patients typically present with symptoms which may include abdominal, shoulder or back pain, loss of appetite, weight loss, jaundice, and occasionally a palpable mass.The typical treatment is surgical removal of the tumor. When the tumor cannot be removed surgically or when there is distant spread, other therapies may be used to slow down the spread of the disease. There is no standard regimen so non-surgical therapy varies from patient to patient. These therapies are not typically curative but may shrink tumors for a period of time. Check out FCF’s FAQ page for a discussion of various treatments for fibrolamellar.
- Fibrolamellar strikes males and females alike
- Risk factors for fibrolamellar remain unidentified
- Fibrolamellar effects primarily teens and young adults (although cases as young as 2 and as old as 74 are known)
- Currently, there are no effective treatment options other than liver resection surgery which can be curative before metastatic spread. Because the liver is the only organ which can regenerate itself, removal of diseased portions of the liver do not leave the patient with compromised liver function due to decreased liver size. Liver function typically returns to normal within 2 to 3 weeks and liver size within 3 to 6 months.
- The fibrolamellar variant of hepatocellular carcinoma differs from the “classical” form of hepatocellular carcinoma in several important aspects including:
- Most patients with fibrolamellar do not have underlying cirrhosis of the liver
- Hepatitis B infection is very uncommon in patients with fibrolamellar
- Serum levels of alpha-fetoprotein are usually not elevated in patients with fibrolamellar
- Initial diagnosis generally comes from symptoms arising with advanced disease
- There could be a link between fibrolamellar and gynecomastia, in that the hormonal influence of the fibrolamellar cancer cells cause the gynecomastia. (Gynecomastia is a condition of pubescent boys/men where they show excessive breast tissue.)
- Fibrolamellar was first identified in the 1950’s and the disease was named by John Craig, MD who is on the Board of Directors of FCF and heads up the Grant Review Committee.