National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Fibrolamellar carcinoma


Other Names:
Fibrolamellar hepatocellular carcinoma; Eosinophilic hepatocellular carcinoma with lamellar fibrosis; Polygonal cell hepatocellular carcinoma with fibrous stroma; Fibrolamellar hepatocellular carcinoma; Eosinophilic hepatocellular carcinoma with lamellar fibrosis; Polygonal cell hepatocellular carcinoma with fibrous stroma; Hepatocellular carcinoma with increased stromal fibrosis; Eosinophilic glassy cell hepatoma; Fibrolamellar oncocytic hepatoma; FL-HCC; FHCC; Fibrolamellar hepatocarcinoma See More
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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.[1][2][3]
Last updated: 4/23/2015
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:[1][2][4][5]
  • Abdominal pain.
  • Loss of appetite.
  • Weight loss.
  • Malaise.
  • Jaundice.
  • Nausea and/or vomiting.
  • A palpable liver mass found on a physical exam.

Other signs and symptoms that have been reported less commonly include:[1][4]

Last updated: 4/13/2018
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.[1][2][3]

Recent research suggests that a deletion on chromosome 19 may play a key role in the formation of FLC.[6] 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: 4/22/2015
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.[1][2]

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: 4/22/2015
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).[1][2]

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: 4/23/2015
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.[2][1] In fact, reported 5-year survival rates range from 51 to 70% following liver resection.[7][8][9][2]

Unfortunately, the prognosis is less favorable in people with unresectable disease. The median survival of these patients is approximately 14 months.[1]
Last updated: 4/23/2015

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Fibrolamellar carcinoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
  • 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.

Patient Registry

  • A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Fibrolamellar carcinoma. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

    Registries for Fibrolamellar carcinoma:
    Fibrolamellar Registry
     

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

  • Good Days provides help to patients with life-altering conditions. Assistance includes help with the cost of medications and travel.

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Fibrolamellar carcinoma. Click on the link to view a sample search on this topic.

News

Other Conferences

  • The National Institute of Diabetes and Digestive and Kidney Diseases, National Cancer Institute, National Institute of Biomedical Imaging and Bioengineering, Department of Veteran Affairs, and Fogarty International Center sponsored a conference on hepatocellular carcinoma on April 1-3, 2004 to discuss the screening, diagnosis, and management of this type of cancer. You can view the agenda as well as a list of speakers by clicking here.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. Michael A Choti, MD, MBA, FACS. Fibrolamellar Carcinoma. Medscape Reference. August 2015; http://emedicine.medscape.com/article/278354-overview.
  2. Lim II, Farber BA, LaQuaglia MP. Advances in fibrolamellar hepatocellular carcinoma: a review. Eur J Pediatr Surg. December 2014; 24(6):461-466.
  3. Arief Suriawinata, MD. Pathology of malignant liver tumors. UpToDate. November 2014; Accessed 4/22/2015.
  4. What is Fibrolamellar?. Fibrolamellar Cancer Foundation. http://fibrofoundation.org/learn/what-is-fibro/. Accessed 4/13/2018.
  5. Chagas AL, Kikuchi L, Herman P, et al. Clinical and pathological evaluation of fibrolamellar hepatocellular carcinoma: a single center study of 21 cases. Clinics (Sao Paulo). January, 2016; 70(3):207-213. https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/26017653/.
  6. Honeyman JN, Simon EP, Robine N, Chiaroni-Clarke R, Darcy DG, Lim II, Gleason CE, Murphy JM, Rosenberg BR, Teegan L, Takacs CN, Botero S, Belote R, Germer S, Emde AK, Vacic V, Bhanot U, LaQuaglia MP, Simon SM. Detection of a recurrent DNAJB1-PRKACA chimeric transcript in fibrolamellar hepatocellular carcinoma. Science. February 2014; 343(6174):1010-1014.
  7. Darcy DG, Malek MM, Kobos R, Klimstra DS, DeMatteo R, La Quaglia MP. Prognostic factors in fibrolamellar hepatocellular carcinoma in young people. J Pediatr Surg. Jan 2015; 50(1):153-156.
  8. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg. December 2012; 215(6):820-830.
  9. Eggert T, McGlynn KA, Duffy A, Manns MP, Greten TF, Altekruse SF. Fibrolamellar hepatocellular carcinoma in the USA, 2000-2010: A detailed report on frequency, treatment and outcome based on the Surveillance, Epidemiology, and End Results database. United European Gastroenterol J. 2013 Oct;1(5):351-7. October 2013; 1(5):351-7.