Summary about Disease
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare form of liver cancer that primarily affects adolescents and young adults, although it can occur at any age. It differs from typical hepatocellular carcinoma (HCC) in its pathology, patient demographics, and often has a slightly better prognosis. The tumor is characterized by distinctive fibrous bands arranged in parallel lamellae that surround the cancerous liver cells (hepatocytes). It also often doesn't involve underlying liver disease like cirrhosis.
Symptoms
Symptoms of FL-HCC are often vague and can be similar to other liver conditions. They may include:
Abdominal pain or a feeling of fullness
A mass or lump felt in the abdomen
Fatigue
Unexplained weight loss
Jaundice (yellowing of the skin and eyes), though this is less common than in other liver cancers
Nausea and vomiting
Loss of appetite
Ascites (fluid buildup in the abdomen)
Shoulder Pain
Causes
The exact cause of FL-HCC is unknown in many cases. A gene fusion involving DNAJB1-PRKACA is frequently found in FL-HCC tumors, suggesting a key role in the development of the disease. However, this fusion isn't present in every case, indicating that other factors may also be involved. Unlike typical HCC, it is typically not associated with underlying liver diseases such as cirrhosis, Hepatitis B or C, or alcohol abuse.
Medicine Used
4. Medicine used There is no single standard drug regimen for FL-HCC. Treatment strategies depend on the stage of the disease, the overall health of the patient, and the availability of clinical trials.
Surgery: If the tumor is localized and resectable (can be completely removed), surgical resection is the primary treatment option and offers the best chance for long-term survival.
Liver Transplant: In some cases, a liver transplant may be considered if the tumor is unresectable or if there is underlying liver dysfunction.
Chemotherapy: Chemotherapy may be used to shrink the tumor before surgery (neoadjuvant therapy) or to treat cancer that has spread to other parts of the body (metastatic disease). Common chemotherapy drugs used include cisplatin, doxorubicin, and fluorouracil.
Targeted Therapy: Some targeted therapies that target specific molecules involved in cancer growth and spread may be used. Sorafenib and Lenvatinib can be used.
Clinical Trials: Participating in clinical trials is encouraged, as they may offer access to new and promising treatments.
Is Communicable
No, FL-HCC is not communicable. It is not an infectious disease and cannot be spread from person to person.
Precautions
Since the cause is largely unknown, there are no specific precautions that can definitively prevent FL-HCC. However, maintaining a healthy lifestyle, including a balanced diet, regular exercise, and avoiding excessive alcohol consumption, is generally recommended for overall health and may indirectly reduce cancer risk. Early detection through regular checkups and prompt evaluation of any concerning symptoms is important.
How long does an outbreak last?
FL-HCC is not an infectious disease causing an "outbreak." It is a type of cancer that develops over time. If left untreated, it will progress until fatal.
How is it diagnosed?
Diagnosis of FL-HCC typically involves a combination of:
Physical Examination and Medical History: The doctor will assess symptoms and risk factors.
Blood Tests: Liver function tests, including alpha-fetoprotein (AFP), are usually performed. AFP levels are often normal or only slightly elevated in FL-HCC, which helps distinguish it from typical HCC. Other tumor markers such as CA19-9 and neurotensin can be elevated
Imaging Studies:
Ultrasound: Can detect liver masses.
CT Scan: Provides detailed images of the liver and surrounding structures, helping to assess the size, location, and extent of the tumor.
MRI: Offers excellent soft tissue contrast and can help differentiate FL-HCC from other liver lesions. MRI can also provide information about the blood supply to the tumor.
Liver Biopsy: A small sample of liver tissue is taken and examined under a microscope to confirm the diagnosis and determine the specific type of cancer. The presence of distinctive fibrous bands and large hepatocytes with abundant cytoplasm is characteristic of FL-HCC.
Genetic Testing: Genetic testing of the tumor tissue can identify the DNAJB1-PRKACA fusion gene.
Timeline of Symptoms
9. Timeline of symptoms The timeline of symptoms can vary considerably.
Early Stages: The disease can be asymptomatic (without symptoms) in the early stages.
Gradual Onset: Symptoms usually develop gradually over weeks or months.
Progression: As the tumor grows, symptoms become more pronounced. Pain becomes more persistent, fatigue increases, and weight loss becomes more significant.
Late Stages: In advanced stages, complications such as jaundice, ascites, and liver failure may occur.
Important Considerations
Rarity: Due to the rarity of FL-HCC, diagnosis can be delayed.
Younger Patients: It predominantly affects younger individuals, which can present unique challenges in treatment and management.
Expert Consultation: It's crucial to seek care at a specialized cancer center with experience in treating liver cancers, especially rare subtypes like FL-HCC.
Prognosis: If the tumor can be completely removed, prognosis can be more favorable compared to other advanced liver cancers.
Clinical Trials: Patients are encouraged to explore clinical trial options.
Psychosocial Support: Coping with a cancer diagnosis, especially in younger patients, requires strong psychosocial support for both the patient and their family.