Fibrolamellar Hepatocellular Carcinoma
Fibrolamellar hepatocellular carcinoma (FHCC) is a rare form of hepatocellular carcinoma (HCC) that typically affects young adults and is characterized, under the microscope, by laminated fibrous layers interspersed between the tumour cells. Approximately 200 new cases are diagnosed worldwide each year.
The list of signs and symptoms mentioned in various sources for fibrolamellar hepatocellular carcinoma (variant) includes the 12 symptoms listed below:
- Asymptomatic in early stages
- Abdominal fullness
- Abdominal pain
- Abdominal distension
- Weight loss
- Jaundice due to obstruction
- Enlarged male breasts
Note that Hepatocellular carcinoma (fibrolamellar variant) symptoms usually refers to various symptoms known to a patient, but the phrase Hepatocellular carcinoma (fibrolamellar variant) signs may refer to those signs only noticable by a doctor.
Due to lack of symptoms, until the tumor is sizable, this form of cancer is often advanced when diagnosed. Local symptoms may include a palpable liver mass.
FHCC often does not produce alpha fetoprotein (AFP), a widely used marker for conventional hepatocellular carcinoma. However, FHCC is associated with elevated neurotensin levels.
In FHCC, plasma neurotensin and serum vitamin B12 binding globulin are commonly increased and are useful in monitoring the disease and detecting recurrence.
FHCC has a high resectability rate, i.e. it can often be surgically removed. Liver resection is the optimal treatment and may need to be performed more than once, since this disease has a very high recurrence rate. Due to such recurrence, periodic follow-up medical imaging (CT or MRI) is necessary.
As the tumor is quite rare, there is no standard chemotherapy regimen.
The survival rate for fibrolamellar HCC largely depends on whether (and to what degree) the cancer has metastasized, i.e. spread to the lymph nodes or other organs. Distant spread (metastases), significantly reduces the median survival rate.