Clinical features of 20 patients with curatively resected biliary neuroendocrine tumours

Abstract

Background: Neuroendocrine tumours very rarely occur in the biliary tract; information about them is limited.

Aims: To present the clinical characteristics and prognosis of curatively resected biliary neuroendocrine tumours.

Methods: Review of medical records dated between 2000 and 2010 of 20 patients from three medical centres with biliary neuroendocrine tumour based on curative resection.

Results: Based on the World Health Organization 2010 classification, five and one patients had neuroendocrine tumour grades 1 and 2, seven had neuroendocrine carcinoma, and seven were diagnosed with mixed adenoneuroendocrine carcinoma. The locations were the following: seven in the gallbladder, four in the extrahepatic bile duct, and nine in the ampulla of Vater. Lymph node and hepatic metastases were noted in 11 and 4 patients, respectively. Fourteen patients experienced recurrence; most had recurrence in the liver. Patients with neuroendocrine tumour grade 1 had a lower rate of recurrence compared to others (p=0.001). The median disease-free and overall survival times were 5.8 (0.4-53.6) and 13.7 (1.9-102.1) months for all four subtypes. However, the median disease free and overall survival rates of neuroendocrine tumours were significantly longer than those of neuroendocrine carcinomas or mixed adenoneuroendocrine carcinoma.

Conclusions: Patients with biliary neuroendocrine tumour showed extremely different clinical outcomes according to histopathologic subtypes by World Health Organization 2010 classification.