The diagnosis and treatment of cholangiocarcinoma.

BACKGROUND: Cholangiocarcinoma (CCA) is the second most common primary hepatic tumor in Germany, with about 3500 new cases per year. In recent years, its prognosis has improved because of wider resections and the establishment of local treatment and chemotherapy in the palliative situation.

METHODS: This review is based on pertinent articles that were retrieved by a selective literature search in the PubMed database with the keywords "cholangiocarcinoma AND diagnostic OR therapy." Articles in English or German published up to January 2014 were considered.

RESULTS: The sole curative treatment for CCA is surgery, but 40-85% of all patients have recurrent disease even after radical excision. Because of this high recurrence rate, adjuvant treatments are now under intense discussion. For unresectable CCA without distant metastases, small case series have shown that liver transplantation can yield promising survival rates of over 50% at 5 years. For many patients with CCA, however, only palliative treatments can be offered, including endoscopic clearing of the biliary pathways. Because of the low prevalence of the disease, there have been only a few phase 3 studies of palliative chemotherapy for CCA. On the basis of one positive phase 3 study, chemotherapy with gemcitabine and cisplatin is considered the standard and now plays an established role in palliative care.

CONCLUSION: CCA presents a special challenge in gastroenterology, oncology, and visceral surgery because of the difficulty in establishing the diagnosis, local complications in the biliary pathways, and a high recurrence rate after resection. Future studies should address not only the role of adjuvant chemotherapy, but also the efficacy of combined local and systemic treatment.