Intrahepatic cholangiocarcinoma: A diagnostic and therapeutic challenge

Objectives: Intrahepatic cholangiocarcinoma (ICC) is a rare tumor of the liver. The diagnosis and treatment of it are difficult. The present study reviewed the diagnostic pathways and operative results for ICC. Methods: A retrospective analysis was made of the clinical presentation, diagnostic pathways, and operative results of 20 patients with ICC who underwent hepatectomy from 1997 to 2004 in our institute. Results: The patients were predominantly female (female : male = 15:5), and ranged in age from 41 to 74 years (median 59.8). Abdominal pain was the main presenting symptom, and hepatomegaly was the commonest physical sign. Diagnosis relied mainly on ultrasound or computed tomography. Without biopsy, only two patients were correctly diagnosed with ICC before surgery. The median size of the tumor was 6 cm (range 1.6–12 cm). Major hepatectomy was carried out in 80% of patients. The operative mortality and morbidity were 15% and 40%, respectively. The median follow up was 18.3 months. Thirteen patients (65%) had recurrences at one or more sites, including, in decreasing frequency, the liver, abdominal lymph node, lung, bone, or skin. The median survival was 17.3 months and the 1‐year, 3‐year, and 5‐year survival rates were 60%, 45%, and 10%, respectively. Conclusions: The poor survival of patients with ICC was mainly due to the delay in diagnosis and the aggressive nature of the disease. The diagnosis of ICC relies on a high index of suspicion as there is no reliable tumor marker and imaging results are usually inconclusive. Radical hepatectomy remains the only chance of cure for ICC. The role of lymph node dissection, liver transplantation, and adjunctive chemotherapy in the treatment of patients with ICC remains to be determined.