Peripheral cholangiocarcinoma (PCC) constitutes the second most common primary liver cancer. Information is lacking on patients with PCC <40 years old undergoing surgical treatment. The aim of this study was to evaluate the influence of age on surgical treatment of patients with PCC based on reviewing the clinicopathologic features and survival rate of 23 patients with PCC <40 years old who received surgical treatment.

The clinical features of 23 younger patients with PCC (<40 years old) who underwent surgical treatment between 1977 and 2000 were reviewed. Clinical features of 284 patients with PCC >40 years old were used for comparison.

Three hundred seven patients with PCC with an age range between 28 and 93 years (mean 57.2, median 56.0) were investigated. The fiftieth decade was the peak PCC age in the series. Clinical presentations and physical findings were similar between younger and older PCC groups. Similar positive serum carcinoembryonic antigen and carbohydrate antigen 19-9 rates (42.9% and 66.7% vs 41.2% and 74.4%, respectively) and a similar rate of hepatolithiasis associated with PCC were also observed between the 2 groups (43.5% vs 48.9%). Younger patients with PCC tended to show less mucobilia, less papillary-type PCC, and a more advanced stage of tumor compared with older patients with PCC. However, postoperative adjuvant chemotherapy and radiotherapy were used more frequently in the older patients with PCC. Operative morbidity and mortality were similar between the 2 groups (surgical mortality rate 7.8%). Follow-up ranged from 1.0 to 167.6 months (mean 13.0, median 5.7). The 1- and 2-year actuarial survival rates were 6.3% and 0% in the younger PCC group and 31.3% and 15.0% in the older PCC group, respectively. Prognosis was dismal for the younger patients with PCC (P = 0.0008), but they may benefit from hepatic resection.

Younger patients with PCC had a significantly worse survival rate than older patients with PCC. Hepatectomy is rational and may benefit younger patients with PCC.