Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma

Abstract

Objective: To analyze resectability and survival in patients with hilar cholangiocarcinoma according to a proposed preoperative staging scheme that fully integrates local, tumor-related factors.

Summary background data: In patients with hilar cholangiocarcinoma, long-term survival depends critically on complete tumor resection. The current staging systems ignore factors related to local tumor extent, preclude accurate preoperative disease assessment, and correlate poorly with resectability and survival.

Methods: Demographics, results of imaging studies, surgical findings, pathology, and survival were analyzed prospectively in consecutive patients. Using data from imaging studies, all patients were placed into one of three stages based on the extent of ductal involvement by tumor, the presence or absence of portal vein compromise, and the presence or absence of hepatic lobar atrophy.

Results: From March 1991 through December 2000, 225 patients were evaluated, 77% of whom were seen and treated within the last 6 years. Sixty-five patients had unresectable disease; 160 patients underwent exploration with curative intent. Eighty patients underwent resection: 62 (78%) had a concomitant hepatic resection and 62 (78%) had an R0 resection (negative histologic margins). Negative histologic margins, concomitant partial hepatectomy, and well-differentiated tumor histology were associated with improved outcome after all resections. However, in patients who underwent an R0 resection, concomitant partial hepatectomy was the only independent predictor of long-term survival. Of the 9 actual 5-year survivors (of 30 at risk), all had a concomitant hepatic resection and none had tumor-involved margins; 3 of these 9 patients remained free of disease at a median follow-up of 88 months. The rates of complications and death after resection were 64% and 10%, respectively. In the 219 patients whose disease could be staged, the proposed system predicted resectability and the likelihood of an R0 resection and correlated with metastatic disease and survival.

Conclusion: By taking full account of local tumor extent, the proposed staging system for hilar cholangiocarcinoma accurately predicts resectability, the likelihood of metastatic disease, and survival. Complete resection remains the only therapy that offers the possibility of long-term survival, and hepatic resection is a critical component of the surgical approach.

Figure 1. Axial magnetic resonance cholangiopancreatography images of a patient with hilar cholangiocarcinoma. The bile ducts appear white. The left liver is shrunken; its medial extent is indicated by the white arrows. The bile ducts in the left liver are dilated and crowded (white arrowheads), with little interposed liver tissue. The tumor is indicated by the black arrow (C).

Figure 2. Flow diagram showing the results of the initial investigation and surgical findings of all patients in the series. *Seven patients were judged unfit for extended hepatic resection, two because of unexpected cirrhosis and five because of significant underlying coronary artery disease. **Resection of supraduodenal bile duct, cholecystectomy, subhilar lymphadenectomy. +Twenty-three patients had distant metastases (liver, peritoneal cavity, lung, or bone), whereas three had disease in retroperitoneal lymph nodes. ++Twenty-five patients had metastases to N2-level lymph nodes and 22 had distant disease (9 to the liver, 9 to the peritoneum, and 4 to liver and peritoneum).

Figure 3. Survival after resection stratified by margin status. Resection with a negative histologic margin is indicated by the solid line (n = 62; median survival 42 months), and resection with a positive margin is indicated by the dashed line (n = 18; median survival 21 months). P < .0075 by log-rank test.

Figure 4. Survival of all patients stratified by T stage. Solid line indicates T1 tumors (n = 87; median survival 20 months). Dashed line indicates T2 tumors (n = 95; median survival 13 months). Dotted line indicates T3 tumors (n = 37; median survival 8 months). P < .0092 by Cox regression (likelihood ratio test for overall significance with 2 df).