Cystic duct carcinoma: a proposal for a new "working definition"

BACKGROUND. Farrar's criteria for cystic duct carcinoma (histopathological diagnosis of a carcinoma strictly limited to the cystic duct) have practical limitations. We propose new "working definition": a gallbladder tumor, the center of which is located in the cystic duct. PATIENTS AND METHODS. Between 1980 and 2000 we diagnosed cystic duct carcinoma in 31 patients, 28 of whom (90%) had increased serum bilirubin concentrations. Extrahepatic bile duct resection and cholecystectomy were performed in 10 patients; in others, extended right hepatectomy (16), right hepatectomy (3), and liver bed resection (2) were necessary as well for a potentially curative resection. Additional procedures were portal vein resection (10) and pancreatoduodenectomy (7). RESULTS. All tumors were adenocarcinomas. Depth of invasion was T2 in 3 patients, T3 in 12, and T4 in 16. Thirteen patients (42%) had lymph node metastasis. Curative resection was performed in 24 patients (77%). Hospital mortality was 5 of 31 (16%). Actuarial 5-year survival rate excluding hospital deaths was 22%. CONCLUSIONS. The proposed "working definition" avoids the problems associated with Farrar's criteria and describes a distinct patient group with an approximately equal proportion of men and women, advanced T stage, but a lower than expected frequency of lymph node metastasis. It establishes a basis for standard reporting of results.