Todani T, Toki A
Department of Pediatric Surgery, Kagawa Medical University, Japan.
Nihon Geka Gakkai Zasshi. 1996 Aug;97(8):594-8.
Biliary cancer develops in 20-30% of the patients with choledochal cyst and pancreatobiliary malunion. Some bile acid fractions and refluxed pancreatic enxymes into the bile duct is probably responsible for carcinogenesis. Cancer often develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic duct. In cystic dilatation, cancer often occurs in the common bile duct, while in diffuse or non-dilated type it occurs in the gallbladder. Cancer usually occurs in younger patients than does biliary cancer in general population, and the average age is in the 40s. The risk of malignancy in cysts with internal drainage is higher than that in primary cysts, and early removal of the retained cyst should be performed as quickly as possible. Although the prognosis of biliary cancer is usually dismal, aggressive procedures are recently gaining better results than that by conventional methods. The prevention of cancer is the procedure of choice by early excision. Removal of the whole extrahepatic bile duct is necessary, even in case of malunion with no biliary dilatation. Cancer rarely arises in the intrahepatic duct after excisional surgery, due to long standing biliary stricture. Wide anastomosis with ductoplasty should be essential. Cancer also occurs in the remnant duct. Excision of the distal duct in the pancreas is also necessary.
胆管癌在20% - 30%的胆总管囊肿和胰胆管合流异常患者中发生。某些胆汁酸组分以及反流至胆管的胰酶可能是致癌的原因。癌症常发生于肝外胆管和胆囊，很少发生于肝内胆管。在囊性扩张中，癌症常发生于胆总管，而在弥漫性或非扩张型中则发生于胆囊。与一般人群中的胆管癌相比，癌症通常发生于更年轻的患者，平均年龄在40多岁。内引流囊肿的恶变风险高于原发性囊肿，应尽快尽早切除残留囊肿。尽管胆管癌的预后通常很差，但最近积极的手术方法比传统方法取得了更好的效果。癌症预防的首选方法是早期切除。即使在没有胆管扩张的合流异常情况下，也有必要切除整个肝外胆管。由于长期胆管狭窄，切除术后肝内胆管很少发生癌症。进行广泛的吻合和胆管成形术至关重要。癌症也发生于残留胆管。胰腺远端胆管的切除也是必要的。
