A retrospective review of 130 patients with peripheral-type cholangiocarcinomas (PTCC), hilar-type cholangiocarcinomas (HTCC), extrahepatic cholan-giocarcinomas (EHCC), gallbladder cancers (GBCA), and periampullary cancers (PACA), seen at National Cheng Kung University Hospital and Tainan Municipal Hospital from June 1987 to July 1993 was performed. There were 47 (36%) HTCC, 32 (25%) PACA, 24 (19%) PTCC, 17 (13%) GBCA, and 10 (8%) EHCC patients. The distribution is completely different from that reported in western countries. These cancers mainly occur in elderly patients. HTCC and GBCA were predominantly noted in female patients. Biliary cancers in Taiwan were not related to liver fluke infestation, inflammatory bowel disease or hepatitis B virus infection. However, a close association with biliary lithiasis was found. The incidence of gallstones was 67, 39,20,29 and 19% for PTCC, HTCC, EHCC, GBCA and PACA, respectively. The most common presentation for PTCC and GBCA was abdominal pain, or jaundice for HTCC, EHCC and PACA. These symptoms correlate well with the location of the tumors. Among serum tumor markers, the elevation of CA19-9 was most frequent, occurring in 86% of the patients while CA125 and CEA occurred in 47% and 30% of the patients, respectively. During the course of disease, infection developed in 61 % of the patients and was the main cause of death in 25%. Biliary tract infection and sepsis were the two leading manifestations and occurred in 49% and 32% of the patients, respectively. Overall survival was poor except in patients whose tumor could be completely resected.
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