Funabiki T, Matsubara T, Ochiai M, Marugami Y, Sakurai Y, Hasegawa S, Imazu H
Department of Surgery, Fujita Health University School of Medicine, Aichi, Japan.
Keio J Med. 1997 Dec;46(4):169-72. doi: 10.2302/kjm.46.169.
It is well known that the frequency of an associated gallbladder cancer in patients with pancreaticobiliary maljunction (PBM) without congenital choledochal dilation (CCD) is very high, while that of bile duct cancer with CCD is remarkably high, and that of bile duct malignancy without CCD is low. However, recent statistical evaluations have demonstrated that the coincidence rates of gallbladder and bile duct cancer with CCD are 11.5% and 4.6%, respectively, whereas without CCD the rates are 57.1% and 4.1%, respectively. Rates of bile duct cancer with CCD are comparable to those without CCD. We have performed biliary reconstruction after resection of extrahepatic bile ducts along with the gallbladder for PBM patients who had neither CCD nor cancer. Our surgical strategy for these patients without CCD with PBM was assessed from K-ras point mutations and overexpression of p53 protein in the epithelia of the cancerous portions and non-neoplastic portions of the gallbladder and bile duct affected by PBM regardless of choledochal dilatation. The mutation rate in the non-neoplastic gallbladder epithelium without CCD was 80%, that of the bile duct without CCD 57%, not significantly different from the 50% and 40%, respectively, with CCD. The frequency of p53 overexpression in the non-neoplastic bile duct epithelium without CCD was 14%, comparable to the 11% in gallbladder epithelium with CCD. Judging from the statistical data and the molecular biological data, resection of an extrahepatic bile duct with the gallbladder should be the treatment of choice for carcinogenesis prevention.
众所周知，在无先天性胆管扩张（CCD）的胰胆管合流异常（PBM）患者中，伴发胆囊癌的频率非常高，而在有CCD的患者中胆管癌的频率显著高，在无CCD的患者中胆管恶性肿瘤的频率低。然而，最近的统计评估表明，有CCD的胆囊癌和胆管癌的符合率分别为11.5%和4.6%，而无CCD时，该率分别为57.1%和4.1%。有CCD的胆管癌发生率与无CCD的相当。对于既无CCD也无癌症的PBM患者，我们在切除肝外胆管及胆囊后进行了胆道重建。我们从受PBM影响的胆囊和胆管的癌性部分及非肿瘤部分上皮中的K-ras点突变和p53蛋白过表达情况，评估了这些无CCD的PBM患者的手术策略，无论有无胆总管扩张。无CCD的非肿瘤性胆囊上皮中的突变率为80%，无CCD的胆管中的突变率为57%，与有CCD时分别为50%和40%相比无显著差异。无CCD的非肿瘤性胆管上皮中p53过表达的频率为14%，与有CCD的胆囊上皮中的11%相当。从统计数据和分子生物学数据判断，切除肝外胆管及胆囊应是预防癌变的首选治疗方法。
