Fibrolamellar Carcinoma

Fibrolamellar carcinoma is a rare form of primary liver cancer which has traditionally been considered to be a distinct variant of HCC
Not associated underlying liver inflammation, cirrhosis or fibrosis
Affects mainly Caucasians; males and females equally affected
Median age of diagnosis is 33 years;

Conventional HCC is still more common in young patients than fibrolamellar carcinoma

Most common presenting symptoms are: abdominal pain, weight loss and malaise
AFP levels often normal. Liver enzymes may be slightly elevated or normal.
Usually presents as a hypoattenuated, well-defined solitary liver mass on a non-enhanced CT scan, sometimes with a central scar, in an otherwise normal appearing liver (i.e. usually no cirrhosis)
Fine-needle aspiration should not be performed in patients with resectable tumours. Referral to a hepatobiliary surgeon is indicated
Surgical resection or transplantation is the standard of care for eligible patients. Subsequent recurrences or development of metastatic disease occurs in more than half of patients. Median time to recurrence is reported to be between 10 and 33 months
TACE may be useful for patients with unresectable liver-limited disease
Chemotherapy for patients with metastatic disease is not well defined. There have been reports of partial responses to platinum-based regimens