Bile duct carcinoid tumors: an uncommon disease but with a good prognosis?

UNLABELLED: Carcinoid tumors of the biliary tract are exceedingly uncommon. We report 3 cases of primary carcinoid tumors of the biliary tract, which are compared to the reported patients in the literature and to the patients' data with other types of biliary malignancies in our experience.

SETTING

Retrospective analysis in a single academic institution. From a consecutive series of 79 resected tumors of the biliary tract between 1979 and 2002, 3 patients were found to suffer from primary carcinoid tumors (3.8%). There were 2 males and one female patient, with a mean age of 56 years old (range: 46-73), younger than other malignant biliary cancer cases. Clinical presentation included obstructive jaundice in 2 patients. There were no signs of diarrhea or carcinoid syndrome. Location of the tumor included the extrahepatic bile duct in all patients. Two of these patients had a polypoid tumor obstructing the bile duct. The disease was localized in 2 patients, but with liver metastases in the remaining patient. Final diagnosis was achieved on final pathological examination in 2 patients, but preoperative endoscopic biopsies allowed correct diagnosis of nature and tumor staging by octreotide scintigraphy in one patient. Radical resection was achieved in 2 patients with long-term cure at 8.5 and 9.5 years, respectively. A high-risk patient with a small residual liver metastasis after resection is still alive and disease-free under LAR Octreotide SC administration at 17 months postoperatively. The median survival time for patients suffering from carcinoid tumors was 102 months while it was 33 months for patients suffering from cholangiocarcinomas in our experience. Carcinoid tumors of the biliary tract are exceedingly rare. Preoperative diagnosis is uncommon but possible by endoscopic biopsies. Radical resection seems to be associated with a longer survival than classical cholangiocarcinoma.