Introduction:
Fibrolamellar carcinoma (FLC) is a slow-growing tumor in the liver. The causes of FLC are unclear. It affects young adults between 15 to 40 years of age. This cancer does not cause any symptoms initially, but as the tumor grows in size, it causes symptoms like abdominal pain, weight loss, and general weakness.
What Is Fibrolamellar Carcinoma?
Fibrolamellar carcinoma gets its name from its appearance on a microscopic level. It appears as a fibrous band of tissues (tissue comprises a bundle of fibers) arranged in fine, alternately arranged layers of different materials called the lamellar pattern. This cancer is rare, and unlike other types of cancers that occur in a liver with an underlying disease like cirrhosis, this FLC appears in a healthy liver.
What Causes Fibrolamellar Carcinoma?
The causes of fibrolamellar carcinoma are unclear as to why it happens in a healthy liver.
What Are the Symptoms of Fibrolamellar Carcinoma?
The fibrolamellar carcinoma shows no symptoms in the initial phase. As the tumor size increases, it pressurizes the organ, and some symptoms occur.
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Abdominal pain (pain in the stomach region).
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Weight loss.
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General weakness.
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Nausea and vomiting.
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Fever due to hepatic abscess (pus-filled sac in the liver).
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Obstructive jaundice (yellowish discoloration of the skin and eyes due to narrowing or a block in the bile duct) due to compression of the biliary tree (a vessel system that transports secretions from the liver, pancreas, and gallbladder to the duodenum. Duodenum is the first part of the small intestine).
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Gynaecomastia (a condition in which a boy or a male’s chest enlarges and appears like breasts).
How Is Fibrolamellar Carcinoma Diagnosed?
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Physical Examination: A physical examination is performed by the doctor to check for enlarged liver and jaundice.
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Liver Function Tests (LFT): A group of blood tests that involve screening of liver enzymes like alanine aspartate (ASP), alkaline phosphatase (ALP), and alanine transaminase (ALT), and proteins like albumin, globulin, and alpha-fetoprotein in the blood. An increased level of alpha-fetoprotein (AFP) suggests liver cancer.
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Tumor Marker Tests: Tumor marker is a type of protein that is present in blood, urine, and body tissues. The increase in the levels of these tumor markers indicates cancer.
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Ultrasound Abdomen: Ultrasound is an imaging technique that uses high-frequency sound waves to capture images of the internal organs or structures to detect any abnormality.
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CT Scan: Computerized tomography is an imaging technique that contains cross-sectional X-ray images of the internal organs at different angles with the help of a computer. It gives a detailed view of the targeted structure to view any abnormality.
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MRI Scan: This imaging technique uses a combination of magnetic field and radio waves from a computer to capture the internal structure and tissues of the body. It gives a very detailed view of the organ or structure.
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PET Scan: Positron emission tomography (PET) uses a radioactive drug to detect both normal and abnormal metabolic activity. A radioactive substance (a nuclear medicine procedure to treat and find out specific diseases) called radiotracer is injected and shows changes in the metabolism, blood flow, regional chemical components, and absorption. Different markers are used for different imaging purposes.
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Liver Biopsy: A small portion of the liver tissue is removed and visualized under a microscope to check for pathological changes. This is a confirmatory test, and the results reveal the type of cancer.
What Are the Treatment for Fibrolamellar Carcinoma?
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Surgery: Surgical excision of the tumor is the prime treatment.
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Resection: A thin layer of the liver is removed along with the tumor in surgical resection.
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Chemotherapy: Chemotherapy involves administering anti-cancer medications that target and kill cancer cells.
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Embolization Therapy: The blood supply to the tumor is cut off by placing a small gelatin sponge or bead on the vessel that supplies blood to the tumor.
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Liver Transplant: In some cases, the tumor along with the liver is removed, and a whole, healthy liver or a part of the healthy liver is transplanted. Since the liver has a regenerative property (forming new tissues), the liver grows back. A liver transplant will not be recommended if cancer has spread to other organs.
Is Fibrolamellar Carcinoma a Common Liver Cancer?
Fibrolamellar carcinoma is rare. It makes up about one to five percent of other liver cancers.
Does Fibrolamellar Carcinoma Recur?
Recurrence is common in liver resection surgery cases. A second surgery will be performed if the tumor recurs.
Are Any Other Cancer Types Similar to Fibrolamellar Carcinoma?
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Hepatocellular carcinoma (cancer of the liver cells).
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Hepatic hemangiomas (a non-cancerous lesion that consists of blood vessels).
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Hepatocellular adenoma (a non-cancerous liver tumor that is common in females who take birth control pills for a long time).
The confirmatory tests like CT scan, MRI, and liver biopsy help confirm cancer.
Are Hepatocellular Carcinoma and Fibrolamellar Carcinoma Same?
No. Hepatocellular carcinoma occurs in patients with an underlying liver disease like cirrhosis (scarring of liver tissues), whereas fibrolamellar carcinoma occurs in young adults with healthy livers without any underlying liver disease.
Can a Fibrolamellar Carcinoma Patient Survive?
In patients with fibrolamellar carcinoma, if cancer has spread to other organs and resection is not possible, the patient will survive for about 14 months.
What Is the Prognosis of Fibrolamellar Carcinoma?
The prognosis depends on the location of the tumor if it has spread to other organs and complete or incomplete removal of the tumor during the surgery. However, 44 % to 68 % of patients who undergo surgery survive for five years, and two to seven percent of patients who receive chemotherapy or other treatment methods survive for five years.
Can Fibrolamellar Carcinoma Be Prevented?
Fibrolamellar carcinoma cannot be prevented, but the risk can be reduced by following a healthy diet, exercising regularly, maintaining the ideal body weight, and avoiding hepatitis B or C infection.
Is Fibrolamellar Carcinoma Hereditary?
Fibrolamellar carcinoma is a rare type of cancer and occurs in a few people. The hereditary (runs in the family) connection with the diseased patient is unclear.
Conclusion:
Fibrolamellar carcinoma is a rare, slow-growing cancer seen in young adults. The cause of this cancer is unknown as it occurs in individuals with a healthy liver. These tumors were initially mistaken for a unique type of hepatocellular carcinoma. But later studies revealed that this is a different type of cancer occurring in healthy young adults without underlying liver disease. The diagnosis of this cancer is usually made at a delayed time, as the symptoms appear late. Many patients visit the doctor when cancer has spread to other parts. This makes the treatment a little complex. Though this is a serious condition, visiting a doctor if any relevant symptom appears and getting appropriate treatment, and following a healthy lifestyle can prolong the lifespan of the patient.
