Long-term survival of a young woman with peripheral cholangiocarcinoma: A case report

Cholangiocarcinoma is the second most common primary tumor of the Liver aft er hepatocellular carcinoma and accounts for 5 to 25% of primary hepatic ma lignancies. Patients with intrahepatic or peripheral cholangiocarcinoma (IC C) most often present at an advanced stage leading to a poor prognosis. A r eview of the literature has produced only 10 patients who have survived ove r five years. We review the case of a young woman with a large cholangiocar cinoma, who has been disease free for eight years. The patient was treated with a right hepatic lobectomy, and received 4 cycl es of 5-fluorouracil and levamisole postoperatively. Known factors associat ed with longer survival in patients with ICC include lack of evidence of lo cal invasion (i.e. capsular, lymphatic, or vascular), negative margins, muc oblia, and well differentiation of the tumor, as well as the absence of lym ph node metastases. Our patient had negative margins and lymph nodes, and s howed no local invasion. However, no mucobilia was noted, and the tumor was only moderately differentiated. Young age has never been associated with i ncreased survival. ICC remains a relatively uncommon tumor with an insidious onset and late pr esentation contributing to poor survival. Surgical resection remains the on ly therapeutic option. Since few patients are potentially resectable at the time of presentation, efforts at early diagnosis and options for adjuvant therapy are imperative.