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.usclivercancer.org/livercanceroverview.html (bottom of page)
  3. http://www.medicinenet.com/liver_cancer/page15.htm
  4. http://rarediseases.info.nih.gov/GARD/Condition/9396/Hepatocellular_carcinoma_fibrolamellar_variant.aspx/Showall

What We Know

  • 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 7 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 FHC do not have underlying cirrhosis of the liver
    • Hepatitis B infection is very uncommon in patients with FHC
    • Serum levels of alpha-fetoprotein are usually not elevated in patients with FHC
  • 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

What We Have Heard

  • There appears to be a concentration of the disease in Shanghai
  • The number of cases worldwide is increasing
  • There could be a link between FHC and gynecomastia (Gynecomastia is a condition of pubescent boys/men where they show excessive breast tissue. Note: we were informed that it is the hormonal influence of the fibrolamellar cancer cells that cause the gynecomastia)
  • There could be two variants of FHC, one more aggressive than the other (Some cancer cells "push" other cells aside, while some cancer cells "eat through" other cells. Tucker’s cancer cells, which are still alive in laboratories, are very aggressive and are "eating through" cells around them)
  • Environmental factors appear to have some play here as the disease and its markers were not seen until the 1950’s
  • There appears to be a hormonal effect of FHC

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

CONTACT USJOIN