CAMBRIDGE, MD. — An early resection with negative tumor margins remains the only hope for long-term survival in patients with cholangiocarcinoma, Dr. Richard Schulick said at a hepatobiliary update sponsored by Johns Hopkins University.
Worldwide, long-term survival in this disease is only about 3.5%, said Dr. Schulick. But in the United States, carefully selected patients whose early tumors are resected with negative margins can potentially have long-term survival, he said.
Patients typically present with right upper quadrant pain, which may be misdiagnosed as cholecystitis, stones, or pancreatic or bile duct cancer. The common cancer symptoms of weight loss and nausea are often present. Neither carcinoembryonic antigen nor carbohydrate antigen 19–9 are sensitive enough to be used exclusively for diagnosis, “although they can be useful in tracking recurrence,” said Dr. Schulick of Johns Hopkins University, Baltimore.
When assessing resectability, Dr. Schulick considers the biliary, vascular, and parenchymal systems separately. “The questions are, 'Can I preserve a portion of the biliary tree, the vascular in- and outflow of that section of the liver, and will there be enough liver left for the patient to survive?'” he said.
He presented survival data from 564 cases of cholangiocarcinoma treated at Johns Hopkins from 1973 to 2004.
Intrahepatic tumors occurred in 44 patients and were treated with hepatic resection, with or without lymph node removal. These patients had the best long-term survival rates. In the 20 whose tumors had negative margins, 5-year survival was 68%. This dropped to 33% for the nine patients whose tumors had positive margins. Fifteen patients received palliative care; their median survival was only 7 months.
Perihilar tumors occurred in 281 patients. These tumors, located in the hepatic duct bifurcation, are treated by excising the extrahepatic biliary tree, with or without lymph nodes. A hepatic resection and/or caudate lobectomy might be required. Operative mortality is relatively high: 4%–10%, Dr. Schulick said. “This is a reflection of the aggressiveness of the operation.”
Long-term survival was not as good in this group, Dr. Schulick said. Of the 52 patients with margin-negative tumors, only 27% were alive at 5 years. Only 8% of the 121 with margin-positive tumors were alive at 5 years. Most of the patients (108) were not candidates for surgery and received palliative care; their median survival time was 9 months.
Distal tumors, located in the distal common bile duct, occurred in 239 patients. These tumors were treated by pancreaticoduodenectomy or excision of the extrahepatic biliary tree.
Again, long-term survival was not good. Of the 187 patients with margin-negative tumors, 21% were alive at 5 years. Only 6% of the 42 with margin-positive tumors were alive at 5 years. Ten patients received palliative care; their median survival time was 13 months.