Liver Pathology - Fibrolamellar hepatocellular carcinoma Flashcards
What is fibrolamellar hepatocellular carcinoma?
Fibrolamellar hepatocellular carcinoma (FHCC) is a rare form of hepatocellular carcinoma that typically affects young adults
How can FHCC be characterized?
It is characterized, under the microscope, by laminated fibrous layers interspersed between the tumour cells.
What are the most common symptoms of FHCC? How important is serum AFP in FHCC?
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.[1]
FHCC often does not produce alpha fetoprotein (AFP), a widely used marker for conventional hepatocellular carcinoma. However, FHCC is associated elevated neurotensin levels.
What is the main difference of FHCC and conventional HCC when it comes to previous liver disease?
FHCC has a low age of onset (~27 years[2]) when compared to conventional HCC. Also, unlike conventional HCC, patients most often do not have coexistent liver disease.
FHCC generally occurs in young adults without underlying cirrhosis. Compared to conventional HCC, FHCC grows slowly and has better prognosis, probably owing to its high resectability.
What are the microscopic features of FHCC? What are the borders of FHCC like?
The histopathology of FHCC is characterized by laminated fibrous layers, interspersed between the tumor cells. Cytologically, the tumor cells have a low nuclear to cytoplasmic ratio with abundant eosinophilic cytoplasm. Tumors are non-encapsulated, but well circumscribed, when compared to conventional HCC (which typically has an invasive border).
What can be dosed to monitor FHCC recurrence along with imaging analysis?
In FHCC, plasma neurotensin and serum vitamin B12 binding globulin are commonly increased and are useful in monitoring the disease and detecting recurrence.
What is the main treatment for FHCC?
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.
What is the prognosis of FHCC?
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.
