What is Fibrolamellar carcinoma?

Fibrolamellar carcinoma, or FLC, is a rare cancer of the liver that usually grows in teens and adults under 40 years old. This type of cancer is different than other types of liver cancer because it happens in people who have healthy livers.

What is the difference between hepatoblastoma and hepatocellular carcinoma?

Hepatoblastoma occurs most frequently in infants or very young children between the ages of 2 months and 3 years. This is the most common kind of cancer of the liver in children. Hepatocellular carcinoma (HCC) occurs most frequently in children between the ages of 10 and 16 years.

How is Fibrolamellar diagnosed?

How is fibrolamellar cancer diagnosed? For most patients, a diagnosis is made following an ultrasound, CT scan and/or MRI of the abdomen. The imaging will often reveal a large liver mass. The diagnosis of fibrolamellar cancer is then confirmed by a biopsy or following surgery to remove the mass.

Is cholangiocarcinoma the same as hepatocellular carcinoma?

Whereas hepatocellular carcinomas develop by malignant transformation of hepatocytes, cholangiocarcinomas arise from the small intrahepatic bile duct epithelium.

What does Fibrolamellar mean?

Fibrolamellar (fibro-la-mel-lar) carcinoma (FLC), also known as fibrolamellar hepatocellular carcinoma, is a rare liver cancer that primarily occurs in adolescents and young adults who have no history of liver disease.

Is fibrolamellar carcinoma curable?

The main treatment option for fibrolamellar carcinoma is surgical removal (resection) of the tumor. Generally, liver resection has proven the most effective method in treating individuals with fibrolamellar carcinoma. The surrounding lymph nodes are also removed because of the risk of the cancer spreading.

What is the survival rate of hepatoblastoma?

Hepatoblastoma Prognosis Survival rates have reached 80-90 percent. This rate depends upon staging at diagnosis. Patients with fully resected tumors have a greater than 85 percent chance of survival. The rate is about 60 percent for those with metastatic disease that responds to chemo.

What is the meaning of hepatoblastoma?

Hepatoblastoma is a very rare cancerous (malignant) tumor that starts in the liver. Symptoms include a lump (mass), swelling, or pain in the belly (abdomen).

Can you survive fibrolamellar carcinoma?

Patients with unresectable metastatic fibrolamellar carcinoma have a median survival of 14 months. The population-based relative survival of patients with fibrolamellar carcinoma in the United States is 73% at 1 year and 32% at 5 years.

What is hepatic cholangiocarcinoma?

Cholangiocarcinoma, also known as bile duct cancer, is an uncommon form of cancer that occurs mostly in people older than age 50. Cancer that occurs in the parts of the bile ducts that are within the liver is called intrahepatic cholangiocarcinoma (ICC), also known as intrahepatic bile duct cancer.

How rare is Fibrolamellar?

Fibrolamellar carcinoma is an ultra rare form of cancer. It affects both men and women and affects approximately 1 in 5,000,000 people in the general population. Fibrolamellar carcinoma occurs with greater frequency among young adults with a median age of diagnosis of 25.

How does fibrolamellar hepatocellular carcinoma ( HCC ) look like?

Fibrolamellar carcinomas often do not produce alpha fetoprotein (AFP) 5. Fibrolamellar HCCs typically are single large tumors with dense fibrotic bands forming a central scar (seen in ~75% of cases) 1, which make it resemble focal nodular hyperplasia (FNH). A few small calcifications are seen in 35-65% of cases 1 .

Can a focal nodular hyperplasia cause fibrolamellar HCC?

Although focal nodular hyperplasia had been proposed as a causative factor for fibrolamellar HCC, no causal relationship has been found [ 6 ]. Fibrolamellar HCC may rarely coexist with other hepatic tumors, such as HCC, focal nodular hyperplasia (FNH), and cholangiocarcinoma [ 7 – 10 ].

How does a fibrolamellar carcinoma look like?

Fibrolamellar carcinomas often do not produce alpha fetoprotein (AFP) 5. Fibrolamellar HCCs typically are single large tumors with dense fibrotic bands forming a central scar (seen in ~75% of cases) 1, which make it resemble focal nodular hyperplasia (FNH).

How old is the average person with fibrolamellar HCC?

The average age at presentation of fibrolamellar HCC is 25 years compared with 65 years for HCC [ 2 – 4 ]. Fibrolamellar HCC does not show any sex predilection, whereas conventional HCC is more common in men.