不可切除的肝门部胆管癌的治疗进展

肝门部胆管癌（hCCA）是位于二级肝管与胆囊管开口之间的胆管上皮起源的恶性肿瘤,又称为Klatskin瘤。手术是hCCA唯一潜在的治愈性方法。切缘性质是行切除手术的患者获得长期生存最关键的因素,而作为胆管癌最常见的亚型,hCCA的病理学类型多为低-中分化腺癌,侵袭能力强,恶性程度高,早期症状不明显且无有效的检出手段,所以多数患者在诊断时已处于疾病晚期,丧失手术指征。虽然新辅助放化疗联合原位肝移植已经被证实是部分局部晚期不可切除的hCCA的有效治疗方式,但纳入标准严格,肝源短缺,部分患者在等待肝源的期间肿瘤进展,失去移植条件,因此该治疗方式无法成为大多数患者的治疗方案。传统的放化疗虽然在一定程度上延长了不可切除hCCA患者的生存时间,但其治疗效果始终有限。有研究表明新辅助放化疗能够将部分不可切除的hCCA降期为可切除,提高R₀切除率,但相关数据较少且陈旧,缺乏可信度。随着科技的进步,包括立体定向放疗、三维适行放疗、放射粒子植入等在内的新型放疗技术和光动力疗法的出现,hCCA的局部治疗进入了更加精准的时代。近年来,随着基因检测的发展和对肿瘤微环境的深入研究,在分子生物学层面抑制肿瘤进展是各种实体肿瘤研究的热门方向,针对不同靶点的靶向药物、免疫检查点抑制剂（PD1/PD-L1抗体、CTLA4抗体）层出不穷,并取得了突飞猛进的进展,为不可切除的hCCA的治疗提供了新的方向。但就目前的研究来看,靶向治疗、免疫治疗虽然在肝内胆管癌的治疗中取得了相当不错的成绩,但是在hCCA治疗中的表现仍令人不满意。不可切除的hCCA的单一治疗效果较差,多种治疗方式联合治疗是当前研究的重点。本文主要综述不可切除的hCCA的治疗进展及新辅助治疗在实现R₀切除方面的可行性,旨在为此类患者的治疗提供一定的参考。     

Multimodal treatment strategies for advanced hilar cholangiocarcinoma

Cholangiocarcinoma (CCA) is the second most common primary malignancy of the liver arising from malignant transformation and growth of biliary ductal epithelium. Approximately 50–70 % of CCAs arise at the hilar plate of the biliary tree, which are termed hilar cholangiocarcinoma (HC). Various staging systems are currently employed to classify HCs and determine resectability. Depending on the pre-operative staging, the mainstays of treatment include surgery, chemotherapy, radiation therapy, and photodynamic therapy. Surgical resection offers the only chance for cure of HC and achieving an R0 resection has demonstrated improved overall survival. However, obtaining longitudinal and radial surgical margins that are free of tumor can be difficult and frequently requires extensive resections, particularly for advanced HCs. Pre-operative interventions may be necessary to prepare patients for major hepatic resections, including endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography, and portal vein embolization. Multimodal therapy that combines chemotherapy with external beam radiation, stereotactic body radiation therapy, bile duct brachytherapy, and/or photodynamic therapy are all possible strategies for advanced HC prior to resection. Orthotopic liver transplantation is another therapeutic option that can achieve complete extirpation of locally advanced HC in judiciously selected patients following standardized neoadjuvant protocols.     

Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma

OBJECTIVE: Compare survival after neoadjuvant therapy and liver transplantation with survival after resection for patients with hilar CCA. SUMMARY BACKGROUND DATA: We developed a protocol combining neoadjuvant radiotherapy, chemosensitization, and orthotopic liver transplantation for patients with operatively confirmed stage I and II hilar CCA in 1993. Since then, patients with unresectable CCA or CCA arising in the setting of PSC have been enrolled in the transplant protocol. Patients with tumors amenable to resection have undergone excision of the extrahepatic duct with lymphadenectomy and liver resection. METHODS: We reviewed our experience between January 1993 and August 2004 and compared patient survival between the treatment groups. RESULTS: Seventy-one patients entered the transplant treatment protocol and 38 underwent liver transplantation. Fifty-four patients were explored for resection. Twenty-six (48%) underwent resection, and 28 (52%) had unresectable disease. One-, 3-, and 5-year patient survival were 92%, 82%, and 82% after transplantation and 82%, 48%, and 21% after resection (P = 0.022). There were fewer recurrences in the transplant patients (13% versus 27%). CONCLUSIONS: Liver transplantation with neoadjuvant chemoradiation achieved better survival with less recurrence than conventional resection and should be considered as an alternative to resection for patients with localized, node-negative hilar CCA.     

Current status of liver transplantation for cholangiocarcinoma

Cholangiocarcinoma (CCA) is the second most common liver cancer with a median survival of 12-24 mo without treatment. It is further classified based on its location into intrahepatic CCA (iCCA), perihilar CCA (pCCA), and distal CCA. Surgical resection is the mainstay of treatment, but up to 70% of these tumors are inoperable at the time of diagnosis. CCA was previously an absolute contraindication for liver transplantation (LT) due to poor outcomes primary due to early recurrent disease. However, improvement in patient selection criteria and neoadjuvant treatment protocols have improved outcomes for inoperable pCCA patients with recent studies reporting LT may improve survival in iCCA. Future advances in the treatment of CCA should include refining patient selection criteria and organ allocation for all subtypes of CCA, determining effective immunotherapies and the evolving role of personalized medicine in patients ineligible for surgical resection or LT. Our article reviews the current status of LT in CCA, along with future directions in managing patients with CCA.     

Long-term outcome of liver resection and transplantation for Caroli disease and syndrome

OBJECTIVE: To assess the preoperative disease characteristics as well as the rate of postoperative complications, patient survival, and course of symptoms after liver resection or orthotopic liver transplantation (OLT) for Caroli disease (CD) or syndrome (CS). SUMMARY BACKGROUND DATA: The clinical course of monolobar or diffuse CD or CS is often characterized by multiple conservative treatment attempts and interventions with recurrent episodes of cholangitis and a serious reduction in quality of life. The role and effectiveness of surgical treatment is still not well defined. PATIENTS AND METHODS: Between June 1989 and December 2002, we treated 44 consecutive patients with CD or CS who had failure of conservative treatment before and were referred for surgical intervention. Demographic and clinical data, operative procedures and related morbidity, course of symptoms, and long-term follow-up were reviewed. Four patients with palliative resection for cholangiocarcinoma and incidental diagnosis of CD were excluded from the analysis. RESULTS: Twenty-two women and 18 men had a median period of 26.5 months from onset of symptoms to surgical therapy. Their median age at therapy was 49 years and 80% of the patients had monolobar disease with a left-right ratio of 2.6 to 1. Thirty-three (82.5%) patients underwent liver resection, while 4 (10%) patients received OLT for diffuse disease. Biliodigestive anastomosis alone was performed in 3 (7.5%) patients with contraindications to OLT. Patients (37.5%) had minor postoperative complications, which were treated conservatively, while 2 (5%) transplanted patients had a reoperation due to intraperitoneal bleeding. After a median follow-up of 86.5 months, we observed a favorable patient and graft survival. Three deaths during follow-up were not related to treatment or disease complications. Follow-up of disease-related symptoms, biliary complications, and antibiotic treatment revealed a significant improvement. CONCLUSION: Our data show that liver resection for monolobar CD or CS and OLT for diffuse manifestations can achieve excellent long-term patient survival with marked symptom relief. Because of life-threatening long-term complications such as biliary sepsis and development of cholangiocarcinoma, timely indication for surgical treatment is crucial.     

Response to Neoadjuvant Chemotherapy Does Not Predict Overall Survival for Patients With Synchronous Colorectal Hepatic Metastases

Objective  We investigated the relation between response to neoadjuvant chemotherapy and overall survival (OS) in patients with colorectal liver metastases (CLM). Background  It has previously been reported that patients with synchronous CLM whose disease progresses while receiving neoadjuvant chemotherapy or who do not receive neoadjuvant chemotherapy experience worse survival than patients whose disease responds to neoadjuvant chemotherapy. Methods  By means of a prospectively maintained surgical database, between 1995 and 2003, we identified 111 patients with a synchronous CLM who received neoadjuvant chemotherapy before hepatic resection. The disease of all 111 patients was deemed resectable, and patients underwent hepatic resection with curative intent. Results  The median OS after liver resection was 62 months, with a median follow-up of 63 months. Median OS was similar between the three study groups classified by response to neoadjuvant chemotherapy (complete or partial response, 58 months; stable disease, 65 months; and disease progression, 61 months; P = .98). By univariate analysis, carcinoembryonic antigen level after liver resection of <5 ng/dL, size of metastatic lesion of ≤5 cm, lymph node–negative primary tumor, and disease-negative margins were associated with improved survival. Patients in the disease progression group had more positive margins and metastases >5 cm in size than patients in the complete or partial response group and the stable disease group. Patients whose tumor progressed but who received postoperative hepatic arterial infusion had a trend toward improved survival compared with those who did not receive hepatic arterial infusion (70% vs. 50% at 3 years, permutation log rank test P = .12). Conclusions  Response to neoadjuvant chemotherapy did not correlate with OS even after controlling for margins, stage of primary tumor, and postoperative carcinoembryonic antigen level. Postoperative salvage treatment may have helped the survival of some patients.     

Role of neoadjuvant chemotherapy in the treatment of multiple colorectal metastases to the liver

BACKGROUND: The role of neoadjuvant chemotherapy for patients with multiple (five or more) bilobar hepatic metastases irrespective of initial resectability is still under scrutiny. The purpose of this study was to compare the outcome of hepatectomy alone with that of hepatectomy after neoadjuvant chemotherapy for multiple bilobar hepatic metastases from colorectal cancer. METHODS: Retrospective data were collected from 71 patients after hepatectomy for five or more bilobar liver tumours. The outcome of 48 patients treated by neoadjuvant chemotherapy followed by hepatectomy was compared with that of 23 patients treated by hepatectomy alone. RESULTS: Patients who received neoadjuvant chemotherapy had better 3- and 5-year survival rates from the time of diagnosis than those who did not (67.0 and 38.9 versus 51.8 and 20.7 per cent respectively; P = 0.039), and required fewer extended hepatectomies (four segments or more) (39 of 48 versus 23 of 23; P = 0.027). Multivariate analysis showed neoadjuvant chemotherapy to be an independent predictor of survival. CONCLUSION: In patients with bilateral multiple colorectal liver metastases, neoadjuvant chemotherapy before hepatectomy was associated with improved survival and enabled complete resection with fewer extended hepatectomies.     

Surgical therapy of proximal extrahepatic bile duct tumors (Klatskin tumors)

Due to their anatomical position, the tendency of early infiltrative growth and their poor prognosis without treatment, klatskin tumors are challenging concerning diagnosis and therapy. In contrast to other tumors of the gastrointestinal tract, for which exact diagnostic and stage dependent therapeutic guidelines could be formulated, clear recommendations for klatskin tumors are missing. Thus, survival rates after local resection, e. g. resection of the bile duct bifurcation alone, show high rates of R1/2 resection and early tumor recurrence. With an additional hepatic resection formally curative resections and long-term survival can be improved. Extended liver resections including the portal vein provide the highest rates of R0 resections for hilar carcinomas of the extrahepatic bile duct. Survival rates after liver transplantation for klatskin tumors are not yet convincing. Promising first results have been reported for the combination of neoadjuvant treatment and liver transplantation and might show future perspectives for the treatment of klatskin tumors.     

Management of hilar cholangiocarcinoma

Hilar cholangiocarcinoma is a rare disease departing from the biliary convergence. It is primarily revealed by the onset of retention jaundice. Only 20%-30% of patients can undergo resection at diagnosis. The only chance for survival for these patients is R0 resection, which requires hepatectomy associated with resection of the common bile duct and pedicle lymph node removal, whatever the classification of the cholangiocarcinoma. No adjuvant treatment has been shown to be effective to date. Palliative treatment is most often based on implanting a biliary stent. Dynamic phototherapy may be beneficial in these situations. Improvements in survival for selected N0 hilar cholangiocarcinoma may be obtained with neoadjuvant treatment with radiochemotherapy followed by liver transplantation.     

Diagnosis and treatment of colorectal liver metastases - workflow

In this review, standards of diagnosis and treatment of colorectal liver metastases are described on the basis of a workshop discussion. Algorithms of care for patients with synchronous / metachronous colorectal liver metastases or locoregional recurrent tumour are presented. Surgical resection is the procedure of choice in the curative treatment of liver metastases. The decision about the resection of liver metastases should consider the following parameters: 1. General operability of the patient (comorbidity); 2. Achievability of an R 0 situation: i. if necessary, in combination with ablative methods, ii. if necessary, neoadjuvant chemotherapy, iii. the ability to eradicate extrahepatic tumour manifestations; 3. Sufficient volume of the liver remaining after resection ("future liver remnant = FLR): i. if necessary, in combination with portal vein embolisation or two-stage hepatectomy; 4. The feasibility to preserve two contiguous hepatic segments with adequate vascular inflow and outflow as well as biliary drainage; 5. Tumour biological aspects ("prognostic variables"); 6. Experience of the surgeon and centre! Extrahepatic disease does not contraindicate hepatectomy for colorectal liver metastases provided a complete resection of both intra- and extrahepatic disease is feasible. Even in bilobar colorectal metastases and 5 or more tumours in the liver, a complete tumour resection has been described. The type of resection (hepatic wedge resection or anatomic resection) does not influence the recurrence rate. Preoperative volumetry is indicated when major hepatic resection is planned. The FLR should be 25 % in patients with normal liver, 40 % in patients who have received intensive chemotherapy or in cases of fatty liver, liver fibrosis or diabetes, and 50-60 % in patients with cirrhosis. In patients with initially unresectable colorectal liver metastases, preoperative chemotherapy enables complete resection in 15-30 % of the cases, whereas the value of neoadjuvant chemotherapy in patients with resectable liver metastases has not been sufficiently supported. In situ ablative procedures (radiofrequency ablation = RFA and laser-induced interstitial thermotherapy = LITT) are local therapy options in selected patients who are not candidates for resection (central recurrent liver metastases, bilobar multiple metastases and high-risk resection or restricted patient operability). Patients with tumours larger than 3 cm have a high local recurrence rate after percutaneous RFA and are not optimal candidates for this procedure. The physician's experience influences the results significantly, both after hepatectomy and after in situ ablation. Therefore, patients with colorectal liver metastases should be treated in centres with experience in liver surgery.     

不同部位胆管癌患者术后生存的影响因素分析

背景与目的 目前,胆管癌（CCA）的首选治疗仍为外科手术,但术后复发率较高,患者生存率低。对CCA患者术后生存影响因素的分析将有助于优化手术策略,从而一定程度上改善患者预后。因此,本研究探讨不同部位CCA患者术后生存的影响因素,为临床提供参考。方法 回顾性分析2011年1月—2020年3月133例行手术治疗的CCA患者临床病理资料,其中,肝内胆管癌（iCCA）58例,肝门胆管癌（hCCA）30例,远端胆管癌（dCCA）45例,分析临床病理特征与患者生存的关系与预后影响因素。结果 iCCA患者术后1、2、3、5年的生存率分别为41.38%、22.41%、8.62%、3.45%,单因素分析显示,iCCA患者的术后生存与合并胆道结石、术前血清白蛋白（ALB）水平、凝血酶时间、CEA、CA125、CA19-9、Child-Pugh分级、肿块最大直径、是否R₀切除、淋巴结转移情况、肿瘤分化程度有关（均P<0.05）;多因素分析显示,术前ALB水平、凝血酶时间、CA19-9、Child-Pugh分级、肿块最大直径及是否R₀切除是iCCA患者术后生存的独立影响因素（均P<0.05）。hCCA患者术后1、2、3、5年的生存率分别为43.33%、20.0%、6.67%、3.33%,单因素分析显示,hCCA患者的术后生存与术前CEA水平、是否R₀切除、淋巴结转移情况、肿瘤分化程度、肿块最大直径、是否侵犯门静脉有关（均P<0.05）;多因素分析显示,是否行R₀切除、淋巴结转移情况、肿瘤分化程度、是否侵犯门静脉及肿块最大直径是hCCA患者术后生存的独立影响因素（均P<0.05）。dCCA患者术后1、2、3、5年的生存率分别为62.22%、31.11%、17.78%、14.29%,单因素分析显示,dCCA患者的术后生存与淋巴结转移情况、肿瘤分化程度有关（均P<0.05）。多因素分析显示,淋巴结转移情况、肿瘤分化程度及是否R₀切除是dCCA患者术后生存的独立影响因素（均P<0.05）。dCCA患者术后生存时间优于iCCA和hCCA患者,但差异无统计学意义（均P>0.05）。结论 不同部位CCA有大致共同的预后影响因素,通过评估这些因素有助于预测CCA预后,完善对CCA患者的分层标准,优化术前和术后治疗方案,延长患者生存时间。     

Central hepatic bisectionectomy with hepatic artery and biliary tracts reconstruction for the patient with nodular type intrahepatic hilar cholangiocarcinoma: A case report

Introduction and importanceA central hepatic bisectionectomy (CHBS) for a hilar cholangiocarcinoma (CCA) is technically challenging because bilateral biliary reconstruction is required after resection. On the other hand, hepatic artery resection and reconstruction in a major liver resection are also technical procedures. In this report, we describe our radical CHBS with hepatic artery and biliary tracts reconstruction for a patient with nodular type intrahepatic hilar CCA.Case presentationA 76-year-old man was referred for further investigation of an incidental hepatic tumor. The hepatic tumor was located from medial sector to anterior sector with encasement of the anterior branch of the right hepatic artery. Based on these findings, we performed a CHBS with right hepatic artery and biliary tracts reconstruction. The histopathological findings revealed that the tumor consisted of moderately differentiated tubular adenocarcinoma with tumor necrosis without a fibrous capsule. In this area, tumors cells had invaded branches of the hepatic vein; however, there was no destructive invasion to the hepatic artery. Consequently, he was diagnosed with a nodular type intrahepatic hilar CCA with pT2aN0M0.Clinical discussionA CHBS is usually performed with the intent of anatomically preserving a patient’s liver as much as possible. Concomitant resection and reconstruction of the hilar vessels and biliary tracts with CHBS is one of the most technically challenging procedures in liver resections.ConclusionA CHBS with hepatic artery and biliary reconstruction may be a promising alternative if expert surgeons perform it on strictly selected patients.     

Orthotopic liver transplantation for unresectable hepatoblastoma: a single center's experience.

BACKGROUND/PURPOSE: Complete surgical resection after chemotherapy is the definitive treatment for hepatoblastoma. However, orthotopic liver transplantation (OLT) is now accepted as a treatment modality for patients with unresectable tumours. The aim of this study was to review a single center's experience of OLT for unresectable hepatoblastoma. METHODS: A retrospective review of 8 patients with unresectable hepatoblastoma who were referred for liver transplantation was conducted. RESULTS: The patients assessed had an age range of 5 to 105 months at presentation; median, 24 months, (5 boys; 3 girls). Two patients have familial adenomatous polyposis, and one has right hemihypertrophy. All 8 patients had received standard chemotherapy according to SIOP (International Society of Pediatric Oncology) protocols. Extrahepatic metastases were found in 3 patients at diagnosis, but none had detectable metastases at the time of OLT. Four patients continued chemotherapy while awaiting OLT. Three patients received whole grafts, and 5 received reduced grafts. The median follow-up period was 22 months (range, 2 to 78 months). Five patients are alive and well, although 1 patient had a second OLT for biliary cirrhosis secondary to biliary stricture at 6 years. Three patients died: one 26 days post OLT of sepsis and two of disease recurrence at 22 months and 70 months posttransplant. The actuarial survival rate is 88% and 65% at 1 and 5 years, respectively, whereas the overall survival rate is 62.5%. CONCLUSION: OLT for unresectable hepatoblastoma without extra hepatic metastases is highly successful with a low recurrence rate.     

Vascular complications after orthotopic liver transplantation after neoadjuvant therapy for hilar cholangiocarcinoma.

Liver transplantation after neoadjuvant chemoradiotherapy has emerged as an effective treatment for patients with localized, node-negative, unresectable hilar cholangiocarcinoma (CCA) or CCA arising in the setting of primary sclerosing cholangitis (PSC). However, concern has arisen regarding the potential for vascular complications due to high-dose neoadjuvant therapy before transplantation. We reviewed our experience with specific aims to determine the incidences of arterial, portal, and hepatic venous complications in patients transplanted for CCA compared with patients who undergo transplantation for other indications, and to describe patient outcome as a result of these vascular complications. We reviewed data for all patients who underwent liver transplantation for CCA between January 1993 and April 2006 and compared the incidences of vascular complications to whole organ and living donor recipient control groups. Sixty-eight patients underwent neoadjuvant therapy and subsequent liver transplantation. Arterial complications arose in 21%; portal venous complications arose in 22%; and overall, 40% developed vascular complications. Late hepatic artery complications occurred more often in living donor recipients transplanted for CCA compared with the living donor control group (P=0.047). Late portal vein complications occurred more often in both whole organ and living donor recipients transplanted for CCA compared with the control groups (P=0.01 and P=0.009). Hepatic venous complications were rare. Patient and graft survival were not different between CCA and control patients. Liver transplantation with neoadjuvant therapy is associated with far higher rates of late arterial and portal venous complications, but these complications do not adversely affect patient and graft survival.     

Current Surgical Management of Hilar and Intrahepatic Cholangiocarcinoma: The Role of Resection and Orthotopic Liver Transplantation

Cholangiocarcinoma (CCA) is a rare but devastating malignancy that presents late, is notoriously difficult to diagnose, and is associated with a high mortality. Surgical resection is the only chance for cure or long-term survival. The treatment of CCA has remained challenging because of the lack of effective adjuvant therapy, aggressive nature of the disease, and critical location of the tumor in close proximity to vital structures such as the hepatic artery and the portal vein. Moreover, the operative approach is dictated by the location of the tumor and the presence of underlying liver disease. During the past 4 decades, the operative management of CCA has evolved from a treatment modality that primarily aimed at palliation to curative intent with an aggressive surgical approach to R0 resection and total hepatectomy followed by orthotopic liver transplantation.     

肝门胆管癌的肝移植治疗：附6例报告

背景与目的 不可切除的肝内胆管癌（hCCA）患者可考虑行肝移植治疗,但在某些方面仍存在争议。因此,本研究总结6例肝移植治疗不可切除hCCA临床疗效,以期为临床诊治提供参考。方法 回顾性分析2015年1月—2021年3月6例在上海交通大学医学院附属瑞金医院行肝移植治疗并规律随访的hCCA患者临床病理资料与生存情况。结果 6例肝移植术式均为原位经典全肝移植,术后病理：肿块型2例,管壁浸润型2例,内生型2例;肿瘤直径>3 cm者4例;周围神经浸润2例;门静脉侵犯3例;肝内转移2例;腺鳞癌1例,腺癌5例。组织学分级3例中分化G2,3例低分化G3;pTMN分期分别为II期1例,IIIa期1例,IIIb期1例,IIIc期2例,IV期1例。随访期间,3例存活,其中2例合并肝硬化失代偿内生息肉型腺癌患者获得长期无瘤生存,1例肿块型腺癌患者术前经新辅助放化疗后目前无瘤存活20个月;死亡3例,其中1例肿块型腺鳞癌患者术后存活18个月,2例管壁浸润型腺癌患者分别存活2个月与24个月。术前减黄操作,术后联用免疫抑制剂和化疗药物对于患者生存期无明显影响。结论 hCCA患者中,对于合并肝硬化的内生息肉型腺癌,且术前排除淋巴结转移者,即使术前不行新辅助放化疗直接行肝移植也可取得较好的疗效,但对有淋巴结转移与神经周围浸润者疗效差。     

Liver transplantation for hilar cholangiocarcinoma: A systematic review

Patients with hilar cholangiocarcinoma (hCCA) have advanced disease at presentation and therefore curative treatment options are limited. Liver transplantation (LT), in the case of unresectable disease, is theoretically an attractive option, as it offers the maximum resection margin and at the same time removes the underlying parenchymal liver disease. In the past years a number of studies have aimed to evaluate to potential beneficial role of neo adjuvant therapy followed by LT for treating patients with unresectable hCCA. The objective of our systematic review was to collect and evaluate long-term outcomes of patients with hCCA undergoing LT. A systematic search of 4 electronic databases (Medline, Scopus, Google Scholar and ClinicalTrails.gov databases) was performed for articles published between January 2000 and May 2019. A total of 13 studies with 698 patients were finally included in the present systematic review. A proportion of 74.4% of patients received combination of chemotherapy and radiation as a part of neoadjuvant therapy. One-, 3- and 5-year overall survival rates ranged greatly among the included studies from 58% to 92%, 31% to 80% and 20% to 74%, respectively. Recurrence rates ranged from 16% to 61%, whilst perioperative mortality ranged from 0% to 25.5%. LT could provide acceptable long-term outcomes in the setting of neoadjuvant chemoradiation and strict patient selection criteria. Taking into account organ shortage, combined with the lack of level I evidence, more prospective randomized trials are needed in order to establish certain indications, rigorous criteria and standardized protocols for LT in hCCA and provide the maximal potential benefits for these patients.     

Obstruction of the extrahepatic bile ducts by tumor debris and/or clots in primary cancer of the liver. Diagnostic and therapeutic problems. Apropos of 9 personal cases and review of the literature

The onset of jaundice in patients with primary liver cancer is sometimes caused by intra ductal biliary system tumor casts, free floating debris or hemobilia. The diagnosis of this complication is now possible by E.R.C.P. or P.T.C. We report 9 cases of unrecognized primary liver tumor revealed by intra biliary system migration of tumor casts or free floating debris. In three patients the diagnosis was operative, but for the last six cases the E.R.C.P. data in addition with U.S., C.A.T. Scan and/or hepatic angiography were helpful in determining the nature of the hepatic and biliary changes, the operability (size, location, extent). A curative surgery was not indicated in our 8 operated patients: liver resection and biliary decompression. With the palliative procedures, bilio enteric by pass (2 cases) T tube (1 case) trans hepatic uni or bilateral tubes. The survey was not longer than 11 months. Three patients died in post operative course. Only 56 patients among 117 observations of the literature have been operated: 10 liver resections including the main hepatic junction in two cases were performed with one hospital death, two patients were alive over 2 years with recurrence and three patients were disease free with a follow up less than one year. The operative mortality with palliative procedures was 34.4%; the one-two years survival was 23%; the two longest survivors lived 41 and 61 months.     

Past and future of biliary atresia

BACKGROUND: With the advent of liver transplantation the outcome of children with biliary atresia (BA) has improved. Is Kasai hepatic portoenterostomy (KHPE) still a valuable option for the treatment of these patients? METHODS: From 1974 to 1998, 77 patients with biliary atresia have been treated at our institution: 50 girls and 27 boys. RESULTS: Seventy-four patients had a KHPE, and 3 patients had no KHPE because of delay in diagnosis. A total of 65 of 74 patients (88%) had undergone KHPE type I, 4 patients (5.4%) KHPE type II, 3 patients (4%) had a Suruga modification, and 2 patients (2.6%) had a portocholecystostomy. Among the 74 patients, 11 were lost to follow-up and their cases were considered failures. Seventeen of our patients are alive at long-term follow-up after KHPE. Among the 77 patients, 33 (43%) had an orthotopic liver transplantation (OLT). Successful KHPE patients underwent transplant at a mean age of 9 years, and KHPE failed at a mean age of 11 months. A total of 25 of 77 (32%) of patients are alive thanks to OLT. In the cohort, the overall survival rate for the KHPE plus OLT is 42 of 77 (55%). Mortality and morbidity rates were more frequent among the younger patients who had early OLT after KHPE failure. In our series, overall survival rate was improved when the patient had a successful KHPE (P < .001). CONCLUSIONS: Kasai hepatic portoenterostomy (KHPE) continues to be a valuable procedure in the treatment of infants with biliary atresia (BA). Successful KHPE permits transplantation at an age at which mortality and morbidity are decreased leading to a better outcome.     

Neoadjuvant chemotherapy as a treatment strategy for multiple bilobar liver metastases from colorectal carcinoma

Hepatic resection remains the only potentially curative therapy for patients with colorectal liver metastases. Because most have multiple bilobar liver metastases, surgical resection is possible in only 25-58% of patients with colorectal liver metastases. Currently, attention is focused on the potential for neoadjuvant chemotherapy to render formerly unresectable patients resectable. The availability of more efficacious chemotherapy agents and an inventive approach to delivery schedules have resulted in an increase in the number of candidates for hepatic resection after neoadjuvant chemotherapy. Although tumor response varies with regimen and/or route of chemotherapy for colorectal liver metastases, with 16-63% tumor response rates, hepatic resection for responders after neoadjuvant chemotherapy gives survival benefits, with 20-48% 5-year survival rates after surgery. Provided that neoadjuvant chemotherapy controls multiple bilobar liver metastases well, aggressive hepatic resection should be considered for patients with those lesions. As a treatment strategy for multiple bilobar liver metastases, neoadjuvant chemotherapy is a useful to increase resection rates and may contribute to the improvement of prognosis in patients with such lesions.