Background/Aims: Hilar cholangiocarcinoma is a rare tumor with a dismal pro
gnosis. Because proximal bile duct cancers are uncommon, outcomes related t
o various therapeutic interventions are not well defined.
Methodology: Between 1985 and 1997, 55 patients with bile duct cancers invo
lving the proximal third of the extrahepatic bile ducts were seen. The mana
gement of patients with resectable and unresectable disease was retrospecti
vely reviewed. All but four patients were followed until the time of death.
Results: Forty patients underwent laparotomy following preoperative assessm
ent of extent of disease and 19 patients (35%) ultimately underwent resecti
on with curative intent. Survival was significantly longer in patients who
underwent resection (2-year survival 47% vs. 18%; P = 0.027). Of those pati
ents whose disease was resected, 11 patients received adjuvant radiotherapy
. Survival for this group was not significantly different from that seen in
patients who did not receive adjuvant radiotherapy. Similarly, in patients
with unresectable disease, administration of radiotherapy was not associat
ed with an improved outcome.
Conclusions: Locoregional extent of disease is the greatest problem in case
s of proximal bile duct cancers. Resection provides the best hope for long-
term survival, but new adjuvant strategies are needed.