"Natural history" of unresected cholangiocarcinoma: patient outcome after noncurative intervention.

OBJECTIVE: To clarify the "natural history" of unresected cholangiocarcinoma.
DESIGN: We reviewed the outcome in 103 patients with biopsy-proven cholangiocarcinoma managed at our institution during a 5-year period (1980 through 1984) who did not undergo curative resection.
MATERIAL AND METHODS: The study group of 56 men and 47 women had initial manifestations of jaundice (71%), abdominal pain (49%), and weight loss (44%). The histopathologic features were confirmed by operation in 73 patients, percutaneous biopsy in 16, endoscopy in 3, or autopsy in 11. The primary tumor site was the common hepatic duct in 27%, the hepatic bifurcation in 27%, the common bile duct in 26%, the liver in 13%, and the right or left hepatic duct in 6%. Biopsy-proven metastatic lesions were identified in 70 patients, including 18 with proven nodal involvement. Surgical intervention (N = 57) most commonly consisted of biliary decompression (26%), biliary bypass (16%), or cholecystectomy (11%).
RESULTS: The operative mortality was 4% (N = 2). The hospital mortality of medically managed patients was 12%. The survival after the onset of symptoms was 53% at 1 year, 19% at 2 years, and 9% at 3 years. Only four patients (4%) lived more than 5 years. Univariate analysis of all hospitalized patients (N = 90) revealed a survival advantage for women, patients 62 years of age or younger, those with blood group A or O, surgical patients, patients who did not require biliary decompression, and those who received further palliative treatment after any type of biliary decompression. Multivariate analysis showed a survival advantage for patients who underwent surgical exploration and those who had further palliative treatment after either operative or percutaneous biliary decompression.
CONCLUSION: Unresected cholangiocarcinoma is a rapidly fatal process, but early intervention affects the course of the disease and likely prolongs patient survival.