FAQ

FCF’S FAQ’S FOLLOW: THE ANSWERS ARE FROM OUR OWN RESEARCH AND EXPERIENCE, AND DO NOT NECESSARILY REPRESENT PROVEN SCIENTIFIC FACT.  FCF DOES NOT RECOMMEND DOCTORS OR PROMOTE ANY SPECIFIC FORMS OF TREATMENT. FCF ONLY PROVIDES INFORMATION FOR THE PATIENT COMMUNITY TO SHARE WITH THEIR PERSONAL PHYSICIANS.

How is fibrolamellar diagnosed?

Fibrolamellar is difficult to diagnose, primarily because its symptoms are attributed to more common causes. For example, many patients complain of general abdominal pain and fatigue.  For most patients with fibrolamellar, the diagnosis is made after a CT-scan or MRI of the abdomen is performed. Imaging studies will often reveal a large liver mass.  The diagnosis of fibrolamellar is then confirmed by a biopsy or after surgery to remove the mass.

What treatments are there for fibrolamellar?*

  1. Surgery –  to “debulk” the tumor and remove any disease visible to the eye of the surgeon. The only treatments with a track record of curing patients are resection or transplant (if possible.)
  2. Chemotherapy – In certain patients chemo can provide partial or complete responses. The only chemo with published results is the combination of 5fu+interferon. In light of the dearth of evidence supporting the use of any particular treatment doctors will often try a wide variety of treatments on a case by case basis.
  3. Local non-surgical treatment:
     a) cryoablation(freezing the tumor)
     b) radio frequency ablation( killing the tumor with heat)
     c) external beam radiation
     d) embolization-with or w/o chemo(for liver only, blood flow to one side of the liver is blocked and sometimes chemo is infused directly into the liver)
     e) nanoknife (irreversible electroporation), this involves the killing of tumors by making holes in them through the application of electrical current
     f) percutaneous hepatic perfusion- the liver’s blood supply is temporarily disconnected from the body’s circulation and chemo is circulated throughout the liver for a short time.
     g) y90 microspheres- small radioactive beads are introduced into the blood vessels that feed the tumor and irradiate them from within
  4. Immunotherapy:
    a) Checkpoint inhibitors – PD1 inhibitors such as Keytruda (pembrolizumab) or Opdiva(nivolumab) may be beneficial alone or in combination with other checkpoint inhibitors, or local treatment such as radiation or cryoablation
    b) CTLA4 inhibitors such as Yervoy (ipilimumab)

The above list may not be complete.

Click here for a very complete overview of treatments for fibrolamellar written by one of our medical advisors, Dr. Choti, on medscape.com 

Are there any clinical trials for fibrolamellar patients?

For detailed information on clinical trials, click here

What are the causes of fibrolamellar?

It is not presently known. Some suggest an environmental link, yet here is no firm evidence pointing to this cause. Recent research has shown a distinct mutation (chimera) present in all those with fibrolamellar. Research is continuing to determine if this chimera plays a role in a patient developing fibrolamellar.

How aggressive should follow-up CT scans be, balancing between early diagnosis of recurring disease and radiation exposure?

A CT scan has about 500x the radiation of an xray. MRI’s are magnetic and have no known adverse effects. Each patient’s doctor should determine which is the best scan to use at varying points in time as different scans yield different information.

What doctors are familiar with fibrolamellar?Samreen Raza, M.D., from left, Claire Verschraegen, M.D., Michael Boggs, class of 2013.

Click here for a listing of doctors – surgeons, oncologists, radiologists – whom we know to have treated fibrolamellar. Contact one of the comprehensive cancer centers in the United States for additional names if your area is not listed.

What is the survival rate for fibrolamellar?

Reported survival rates range from 7 to 40 percent five years after diagnosis. Complete surgical removal of the tumor improves this survival rate. Combined with early detection, complete removal of the tumor can also decrease the rate of recurrence.

Patients whose tumors are not removed surgically have an average survival of 12 months, while those who had tumors completely removed survived for an average of 9 years. The Foundation is aware of fibrolamellar patients who remain NED (no evidence of disease) for as long as 20 years after complete removal of the tumor and before any spread of disesase.