Extrahepatic biliary cystadenomas and cystadenocarcinoma. Report of seven cases and review of the literature.

OBJECTIVE: The aim of this investigation was to describe the clinical features, diagnosis, pathologic characteristics, and optimal surgical management for patients with extrahepatic biliary cystadenomas. SUMMARY BACKGROUND DATA: Extrahepatic biliary cystadenomas are rare epithelial neoplasms. The clinical features and optimal surgical management for these lesions have not been defined clearly. The usual presenting symptom is jaundice. These lesions should be considered premalignant and necessitate resection. Sporadic case studies have reported instances of recurrence with local excision. To the authors' knowledge, this study represents the largest collected single series of extrahepatic biliary cystadenomas and reviews previously reported cases.
METHODS: The authors reviewed and reported their institutional experience from 1950 to 1993 in treating seven patients with extrahepatic biliary cystadenomas as well as 19 previously reported cases in the literature.
RESULTS: A strong female predominance (96.3% of patients reviewed) was associated with extrahepatic biliary cystadenomas. Obstructive jaundice was the most common presenting symptom (85%). Abdominal pain occurred in 50% of patients; other symptoms included fever and hemobilia. The most common site of occurrence was the common hepatic duct (32%). Papillary cystadenoma with foci of invasive adenocarcinoma, thus supporting the malignant potential of cystadenomas, occurred in one patient. Local excision from the wall of the bile duct was performed in 18 patients and was associated with 50% recurrence within a mean follow-up of 13 months (range, 4-24 months). No recurrence was reported after formal sleeve resection and bilioenteric reconstruction.
CONCLUSIONS: Extrahepatic biliary cystadenomas can become malignant, and in this study, local surgical excision was associated with a 50% local recurrence rate. Sleeve resection with negative histologic resection margins followed by bilioenteric reconstruction, therefore, is recommended.