早期肝门部胆管癌放疗联合肝胰十二指肠整块切除和原位肝移植后患者长期无瘤存活情况报告

Long-term, tumor-free survival after radiotherapy compining hepatectomy-Whipple en bloc and orthotopic liver transplantation for early-stage hilar cholangiocarcinoma

目的回顾筛查和早期诊断胆管细胞癌和应用全肝及胰十二指肠整块切除-原位肝移植（OLT—Whipple）根治继发于原发性硬化性胆管炎（PSC）的早期肝门部胆管癌的经验。方法采用超声内镜（EUS）、经内镜逆行胰胆管造影（ERCP）并行多级刷片细胞学检查对无症状PSC患者进行筛查。对确诊为胆管癌的患者采用外线柬放疗结合病变局部近距离放射治疗和OLT—Whipple手术进行治疗。结果1988年1月至2001年2月，共对119例PSC患者进行了筛查，并对其中42例运用上述方法进行随访。有8例患者检查出患有胆管癌，其中6例接受了OLT-Whipple手术。在这6例患者中，I期胆管癌4例，Ⅱ期胆管癌2例。这6例患者均接受了OLT—Whipple手术联合外线束放疗和近距离放射疗法。从确诊到实行OLT—Whipple手术的中位时间为144d。1例患者在移植术后55个月死于其他因素，而非肿瘤复发。其他5例无瘤生存达到79、82、108、128、129和145个月。结论对于PSC患者采用ERCP细胞学检查和超声内镜检查可以早期诊断肝门部胆管癌。外放疗和病灶局部放疗联合OLT-Whipple手术整块切除胆道系统有希望获得长期无瘤生存。所有的患者均耐受了这种大范围手术并获得较好的生活质量。该发现支持对并发有PSC的早期肝门部胆管癌患者行OLT—Whipple手术切除整个胆道系统。

Objective To report the experience in surveillance and early detection of cholangioearcinoma （CC） and in using en bloc total hepatectomy-pancreaticoduodenectomy-orthotopic liver transplantation （OLT-Whipple） to achieve complete eradication of early-stage CC complieating primary selerosing cholangitis （PSC）. Methods Asymptomatic PSC patients underwent surveillance using endoscopic ultrasound and endoscopic retrograde cholangiopancreatography （ERCP） with multilevel brushings for cytological evaluation. Patients diagnosed with CC were treated with combined extra-beam radiotherapy, lesion-focused brachytherapy, and OLT-Whipple. Results Between January 1988 and February 2001,42 of 119 PSC patients were followed according to the surveillance protocol. CC was detected in 8 patients, 6 of whom underwent OLT-Whipple. Of those 6 patients, 4 had stage I CC, and 2 had stage U CC. All 60LT-Whipple patients received combined external-beam and brachytherapy radiotherapy. The median time from diagnosis to OLT-Whipple was 144 days. One patient died 55 months post-transplant of an unrelated cause, without tumor recurrence. The other 5 were well without recurrence at 79,82,108,128,129 and 145 months. Conclusions For patients with PSC, ERCP surveillance cytology and intralumenal endoscopic ultrasound examination allow for early detection of CC. Broad and lesion-focused radiotherapy combined with OLT-Whipple to remove the biliary epithelium en bloc offers promising long-term, tumor-free survival. All patients tolerated this extensive surgery well with good quality of life following surgery and recovery. These findings support consideration of the complete excision of an intact biliary tree via OLT- Whipple in patients with early-stage hilar CC complicating PSC.