FAQ: Fibrolamellar


Frequently Asked Questions About Fibrolamellar Hepatocellular Carcinoma

By Dr. Irene Isabel P. Lim

Fibrolamellar is a rare, malignant tumor that arises from the liver. It was first described in 1956 and accounts for only 1% to 5% of all cases of classic hepatocellular carcinoma (HCC). The tumor is typically characterized by a fibrous bands in a unique “lamellar” pattern under the microscope. It is primarily diagnosed in patients from their young teens to late twenties.

Like fibrolamellar, HCC is a malignant tumor of the liver. The similarities, however, stop there. HCC is often associated with chronic inflammation of the liver, either by alcohol abuse or infection by hepatitis B or C. On the other hand, fibrolamellar often occurs in the absence of alcohol or hepatitis infection, with hardly any sign of an inflammatory reaction in the liver. In fact, fibrolamellar patients tend to be younger, while HCC patients are older(1).

Currently, there are no known causes of fibrolamellar. HCC has long been thought to be caused by the chronic inflammation of the liver, but without this inflammatory response, fibrolamellar cannot be attributed to the same cause. Recent research has shown a distinct mutation that may play a key role in the formation of fibrolamellar(2).

Fibrolamellar is rare and accounts for less than one percent of all primary liver cancers(3).

Fibrolamellar is difficult to diagnosis, primarily because its symptoms are attributed to more common causes. Many patients complain of abdominal pain and fatigue, which can be caused by a multitude of things in the pediatric and adolescent population including the flu and a gastrointestinal virus. And unlike HCC, there is no blood test available to raise a doctor’s suspicion that the symptoms may be caused by something other than the flu. For most patients with fibrolamellar, the diagnosis is made after an imaging study such as a CT-scan or MRI of the abdomen is performed, long after all other tests are completed and other causes ruled out. Imaging studies will reveal an often large liver mass.

Surgery to remove the tumor and the surrounding lymph nodes is the standard treatment for fibrolamellar. Lymph nodes are removed as metastases (the spread of the cancer) to the lymph nodes is common. There are no well-studied chemotherapy or other surgical alternatives.

Due to its rare nature, there are no large studies of fibrolamellar. Thus, reported survival rates can range anywhere from 7 to 40 percent at five years. 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 had an average survival of 12 months, while those who had tumors completely removed survived for an average of 112 months(4). It should be noted that the Fibrolamellar Registry is aware of a cohort of fibrolamellar patients who remain NED (no evidence of disease) for as long as 20 years.

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1. Mayo SC, Mavros MN, Nathan H, Cosgrove D, Herman JM, Kamel I, Anders RA, Pawlik TM. Treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma: a national perspective. Journal of the American College of Surgeons. 2014;218(2):196-205. doi: 10.1016/j.jamcollsurg.2013.10.011. PubMed PMID: 24315886.
2. 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. 2014;343(6174):1010-4. doi: 10.1126/science.1249484. PubMed PMID: 24578576.
3. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology. 2004;39(3):798-803. doi: 10.1002/hep.20096. PubMed PMID: 14999699.
4. Stipa F, Yoon SS, Liau KH, Fong Y, Jarnagin WR, D’Angelica M, Abou-Alfa G, Blumgart LH, DeMatteo RP. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006;106(6):1331-8. doi: 10.1002/cncr.21703. PubMed PMID: 16475212.