Fibrolamellar hepatoma (FL-HCC) is an uncommon variant of hepatocellul
ar carcinoma (HCC), distinguished by histopathological features sugges
ting greater differentiation than conventional HCC. However, the optim
al treatment and the prognosis of FL-HCC have been controversial. Foll
ow-up studies are available from 1 year to 27 years, after 41 patients
with FL-HCC were treated with partial hepatectomy (PHx) (28 patients)
or liver transplantation (13 patients). In this retrospective study,
the effect on outcome was determined for the pTNM stage and other prog
nostic factors routinely recorded at the time of surgery, Cumulative s
urvival at 1, 3, 5, and 10 years was 97.6%, 72.3%, 66.2%, and 47.4%. T
umor-free survival at these times was 80.3%, 49.4%, 33%, and 29.3%, Th
e TNM stage was significantly associated with tumor-free survival. Pat
ients with positive nodes had a shorter tumor-free survival than those
with negative nodes (P < .015), Patient survival was most adversely a
ffected by the presence of vascular invasion (P < .05), FL-HCC is an i
ndolently growing tumor of the liver, which usually was diagnosed in o
ur patients at a stage too advanced for effective surgical treatment o
f most conventional HCC. Nevertheless, long-term survival frequently w
as achieved with aggressive surgical treatment. When a subtotal hepate
ctomy could not be performed, total hepatectomy (THx) with liver trans
plantation was a valuable option.