Background/aims: Hilar cholangiocarcinoma is a rare tumor with a dismal prognosis. Because proximal bile duct cancers are uncommon, outcomes related to various therapeutic interventions are not well defined.
Methodology: Between 1985 and 1997, 55 patients with bile duct cancers involving the proximal third of the extrahepatic bile ducts were seen. The management of patients with resectable and unresectable disease was retrospectively reviewed. All but four patients were followed until the time of death.
Results: Forty patients underwent laparotomy following preoperative assessment of extent of disease and 19 patients (35%) ultimately underwent resection with curative intent. Survival was significantly longer in patients who underwent resection (2-year survival 47% vs. 18%; P = 0.027). Of those patients 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 associated with an improved outcome.
Conclusions: Locoregional extent of disease is the greatest problem in cases of proximal bile duct cancers. Resection provides the best hope for long-term survival, but new adjuvant strategies are needed.