What is Fibrolamellar?

Fibrolamellar- (fibro-la-mel-lar) is our abbreviated name for – Fibrolamellar Hepatocellular Carcinoma which is a rare form of liver cancer that usually occurs in young adults who have no history of liver disease. Each year, approximately 200 people are diagnosed with this cancer worldwide. Patients typically present with a palpable abdominal mass but no symptoms, although pain, weight loss and jaundice may occur. The typical treatment is surgical removal of the tumor. When the tumor cannot be removed surgically or when there is distant spread, chemotherapy is used. There is no standard chemotherapy regimen so the chemo cocktail varies from patient to patient.

DEFINITIONS AND DESCRIPTIONS OF FIBROLAMELLAR

  1. http://emedicine.medscape.com/article/278354-overview
  2. http://www.medicinenet.com/liver_cancer/page15.htm
  3. http://rarediseases.info.nih.gov/GARD/Condition/9396/
    Hepatocellular_carcinoma_fibrolamellar_variant.aspx/Showall

Fibrolamellar Facts

  • Fibrolamellar strikes males and females alike
  • Risk factors for fibrolamellar remain unidentified
  • Currently, there are no effective treatment options other than liver resection surgery
  • It effects primarily teens and young adults (although cases as young as 2 and as old as 74 are known)
  • 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
  • Only two organs in the body have an ability to regenerate themselves: the liver and the skin
  • 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

Header Photos:  (L to R) CT scan of fibrolamellar tumor;  fibrolamellar cells under a microscope

CONTACT USJOIN