The Cancer

Fibrolamellar-Hepatocellular Carcinoma (FLL-HCC)

Fibrolamellar-hepatocellular carcinoma (FLL-HCC) is a very rare form of liver cancer that affects young men and women with otherwise normal livers. Because FLL-HCC is so uncommon, there has been limited research and progress toward understanding its cause and how best to treat it.

Memorial Sloan-Kettering Cancer Center is one of the few facilities in the United States with experience caring for patients with FLL-HCC. As part of the Fibrolamellar Hepatocellular Carcinoma Consortium we will also soon be opening a clinical trial testing a new approach to treating the disease.

About Fibrolamellar-Hepatocellular Carcinoma

According to the Surveillance, Epidemiology and End Results (SEER) database, FLL-HCC makes up less than 1 percent of all primary liver cancers. FLL-HCC has features that are distinct from the more common form of primary liver cancer known as hepatocellular carcinoma (HCC).  These are some of these differences.

Age at diagnosis: FLL-HCC is usually diagnosed in adults in their 20s and 30s. It can also appear in some children, adolescents, and older adults. Typical HCC appears only once people enter their 60s and 70s.

Gender: Although HCC is more common in men, FLL-HCC occurs equally in women and men.

Cirrhosis: Scarring of the liver is common in people with HCC, but is extremely rare in people with FLL-HCC.

Alpha-fetoprotein (AFP) levels: Although common in people with HCC, elevated levels of this protein are rare in FLL-HCC.

Risk Factors

Patients who are diagnosed with FLL-HCC do not have the usual risk factors associated with typical hepatocellular carcinoma, such as liver damage or cirrhosis caused by alcohol or viral hepatitis. At this time, we do not know what causes FLL-HCC and no specific risk factors have been identified.


There are no specific symptoms for FLL-HCC. Patients may report pain and/or a mass in the abdomen; feeling bloated, tired, weak, or generally unwell; or having nausea and/or vomiting.  On rare occasions, patients develop jaundice, the symptoms of which include yellowing of the eyes and skin, itching, tea-colored urine, and/or chalk-colored stools. Young male patients may notice breast enlargement.

Diagnosis & Staging

No specific blood tests can be used to diagnose FLL-HCC. Although high levels of specific proteins have been detected in some patients with FLL-HCC, they have not been confirmed as valid markers of the disease. Because of this, FLL-HCC is diagnosed through the use of imaging tools and biopsies.

Imaging Tools

Several imaging tools can be used to help diagnose FLL-HCC and determine its stage (the extent of its spread in the body). Imaging studies play an important part in helping your doctor to plan the best treatment.


An ultrasound is a good screening test for detecting a liver mass or tumor, and it can provide basic information about the tumor and the surrounding liver tissue. A special kind of ultrasound, known as Doppler ultrasound, looks at the portal and hepatic veins, which are major blood vessels of the liver, to determine if the tumor has invaded these structures or if they contain a blood clot. Ultrasounds can also be used to guide the interventional radiologist in obtaining a sample of the tumor (a biopsy).

Triphasic computed tomography scan

A triphasic CT scan is a special type of scan that takes pictures carefully timed with your heartbeat to capture the three phases of blood flow through the liver. Certain characteristics of the liver tumor during these phases can suggest a diagnosis of FLL-HCC. A CT scan also helps our doctors to determine the stage of the cancer; that is, whether it has invaded surrounding structures such as the bile ducts, gallbladder, and blood vessels, or has spread (metastasized) to lymph nodes or other organs outside of the liver. Before the test begins, a contrast material is injected into the bloodstream to improve the resulting image. CT scans can also be used to guide a biopsy.

Magnetic resonance imaging

Patients who are allergic to iodine contrast or who cannot have a CT scan for other reasons can undergo an MRI, which provides information similar to that of a CT scan.

Positron emission tomography

A PET scan is a nuclear medicine study that is often used in conjunction with a CT or MRI to identify metastases (cancer that has spread). Although FLL-HCC can be detected on a PET scan, CT and MRI are the preferred imaging tests for diagnosis and staging.


A biopsy is the most important diagnostic tool for identifying FLL-HCC. Under the guidance of a CT or ultrasound, a needle is inserted into the tumor to withdraw a sample. This technique is called fine needle aspiration (FNA). A pathologist then analyzes the cells under a microscope.

Treatment at Memorial Sloan-Kettering

Although FLL-HCC is a rare disease, Memorial Sloan-Kettering Cancer Center offers several treatment options. Which approach or combination of approaches your doctor recommends will depend on how extensive the tumor is and your general health.


Surgery is the cornerstone of therapy for patients with FLL-HCC. Patients who have tumors that can be removed completely by surgery can survive for a number of years and some patients can be cured.  If FLL-HCC returns, a second surgery is possible in some patients. In rare cases, patients with limited metastases outside of the liver can still undergo surgery to remove as much visible disease as possible. If FLL-HCC has spread widely from the primary tumor site in the liver to other parts of the body, however, surgery is no longer an effective strategy for eliminating the cancer.

Liver Transplantation

Certain patients can undergo a liver transplant but must meet very strict criteria to be eligible.  Transplantation may be an option for patients who are otherwise healthy and who have a limited number of small tumors confined to the liver without any blood vessel involvement. (Blood vessels connected to tumors can allow cancer cells to escape and spread to other parts of the body.)

Hepatic Artery Embolization

Patients who have FLL-HCC that is confined to the liver but who are unable to undergo surgery may benefit from a procedure called hepatic artery embolization. An interventional radiologist inserts a needle and then a small tube into the main artery of the leg. The tube is pushed upward to sit in the main artery that supplies the liver. Tiny plastic beads are then injected to clog the blood supply of the liver tumors. In some cases, the beads are coated with chemotherapy. The procedure can be performed several times.

Systemic Therapy and Investigational Approaches

Systemic treatment options – medications that circulate throughout the body to eliminate cancer cells – are very limited for FLL-HCC. So far, very few clinical trials have included patients with FLL-HCC, and all have been combined with patients who have typical hepatocellular carcinoma.  Patients are sometimes treated with the same drugs that are used to treat typical hepatocellular carcinoma — for example, sorafenib (Nexavar®) — but no clinical trials have shown that this is effective for FLL-HCC.

The Fibrolamellar Hepatocellular Carcinoma Consortium

The Fibrolamellar Hepatocellular Carcinoma Consortium is a collaborative initiative that has been developed to improve treatment options for people with FLL-HCC. Led by Memorial Sloan-Kettering Cancer Center in conjunction with Johns Hopkins University and the University of California, San Francisco, the consortium’s mission is to:

  • promote awareness about FLL-HCC within the oncology community
  • develop new therapies for FLL-HCC
  • pool information on patients with FLL-HCC in order to document trends in diagnosis, treatment, and survival

The consortium will soon be opening a clinical trial testing a novel treatment designed specifically for FLL-HCC. The treatment will target certain cell-signaling pathways that appear to be overactive in this disease. This will be the first clinical trial dedicated to patients with advanced FLL-HCC that cannot be treated with surgery.

In addition, the consortium plans to open a registry to collect information on patients with FLL-HCC. Patients who agree to participate will be asked to donate a blood sample as well as available tumor material. These samples will be banked and used for future research projects on FLL-HCC.