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Abstract：
BACKGROUND: Congenital intrahepatic bile duct dilatation without fibrosis is called Caroli disease (CD), and is called Caroli syndrome (CS) when it has fibrotic and cirrhotic liver morphology. The development of intrahepatic carcinoma is described in both conditions, but the reported incidence varies extensively. Potential risk factors for the malignant transformation were not described. Furthermore, conservative or surgical treatment is performed depending on the extent of cystic malformation, hepatic dysfunction and structural hepatic changes, but little is known about which treatment should be offered to patients with CD or CS and cancer.
OBJECTIVE: To further investigate the malignant transformation in these conditions.
METHODS: A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms \"caroli disease\", \"caroli syndrome\", \"tumor\", \"malignant\", and \"cholangiocarcinoma\". Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence.
RESULTS: Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period.
CONCLUSIONS: Only few retrospective studies reported a low tumor incidence. Despite the high rate of mortality and tumor recurrence, definite surgical treatment should be offered as soon as possible.
OBJECTIVE: To further investigate the malignant transformation in these conditions.
METHODS: A systematic review of the current literature until January 2019 was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. A search using Medline (PubMed) was performed using a combination of Medical Subject Headings terms \"caroli disease\", \"caroli syndrome\", \"tumor\", \"malignant\", and \"cholangiocarcinoma\". Only human studies published in English were used for this systematic review. The following parameters were extracted from each article: year of publication, type of study, number of patients, incidence of malignant tumor, duration of symptoms, age, sex, diagnostics, identification of tumor, surgical therapy, survival and tumor recurrence.
RESULTS: Twelve retrospective studies reporting the courses of 561 patients (53% females) were included in this systematic review. With a mean age of 41.6 years old (range 23 to 56 years old), patients were younger than other populations undergoing liver surgery. Depending on the size of the study population the incidence of cholangiocarcinoma varied from 2.7% to 37.5% with an overall incidence of 6.6%. There were only few detailed reports about preoperative diagnostic work-up, but a multimodal work-up including ultrasound of the liver, computed tomography, magnetic resonance imaging and endoscopic retrograde cholangiopancreatography was used in most studies. Disease duration was variable with up to several years. Most patients had episodes of cholangitis, sepsis, fever or abdominal pain. Tumor detection was an incidental finding of the surgical specimen in most cases because it is currently often impossible to detect tumor manifestation during preoperative diagnostics. Liver resection or liver transplantation was performed depending on the extent of the biliary pathology and additional alterations of the liver structure or function. No postoperative adjuvant chemotherapy was reported, but chemotherapy was administered in selected cases of tumor recurrence. Overall survival rates after one year were low at 36% and a high recurrence rate of up to 75% during the observation period.
CONCLUSIONS: Only few retrospective studies reported a low tumor incidence. Despite the high rate of mortality and tumor recurrence, definite surgical treatment should be offered as soon as possible.
摘要：
背景：无纤维化的先天性肝内胆管扩张被称为Caroli病（CD），当它具有纤维化和肝硬化肝脏形态时，称为Caroli综合征（CS）。在这两种情况下都描述了肝内癌的发展，但报告的发病率差异很大。未描述恶性转化的潜在危险因素。此外，根据囊性畸形的程度进行保守或手术治疗，肝功能障碍和结构性肝变化，但是对于CD或CS和癌症患者应提供哪种治疗方法知之甚少.
目的：进一步研究这些疾病的恶性转化。
方法：根据系统评价和荟萃分析陈述的首选报告项目，对截至2019年1月的现有文献进行系统评价。使用Medline（PubMed）进行搜索，使用医学主题词“caroli疾病”的组合，\"caroli综合征\",\"肿瘤\",“恶性”，和“胆管癌”。只有以英文发表的人类研究被用于本系统综述。从每篇文章中提取以下参数：出版年份，研究类型,患者数量,恶性肿瘤的发病率，症状持续时间，年龄,性别,诊断,肿瘤的鉴定，手术治疗,生存率和肿瘤复发。
结果：12项回顾性研究报告了561例患者（53％为女性）的病程，纳入本系统综述。平均年龄为41.6岁（23至56岁），患者比其他接受肝脏手术的人群年轻.根据研究人群的大小，胆管癌的发病率从2.7％到37.5％不等，总发病率为6.6％。关于术前诊断检查的详细报告很少，而是多模式检查，包括肝脏超声检查，计算机断层扫描,磁共振成像和内镜逆行胰胆管造影术用于大多数研究。疾病持续时间是可变的，长达几年。大多数病人有胆管炎发作,脓毒症,发烧或腹痛。在大多数情况下，肿瘤检测是手术标本的偶然发现，因为目前通常不可能在术前诊断期间检测到肿瘤表现。根据胆道病理的程度以及肝脏结构或功能的其他改变，进行了肝切除或肝移植。无术后辅助化疗报告，但对部分肿瘤复发病例给予化疗。在观察期间，一年后的总生存率为36％，复发率高达75％。
结论：只有少数回顾性研究报告肿瘤发病率较低。尽管死亡率和肿瘤复发率很高，应尽快提供明确的手术治疗。
目的：进一步研究这些疾病的恶性转化。
方法：根据系统评价和荟萃分析陈述的首选报告项目，对截至2019年1月的现有文献进行系统评价。使用Medline（PubMed）进行搜索，使用医学主题词“caroli疾病”的组合，\"caroli综合征\",\"肿瘤\",“恶性”，和“胆管癌”。只有以英文发表的人类研究被用于本系统综述。从每篇文章中提取以下参数：出版年份，研究类型,患者数量,恶性肿瘤的发病率，症状持续时间，年龄,性别,诊断,肿瘤的鉴定，手术治疗,生存率和肿瘤复发。
结果：12项回顾性研究报告了561例患者（53％为女性）的病程，纳入本系统综述。平均年龄为41.6岁（23至56岁），患者比其他接受肝脏手术的人群年轻.根据研究人群的大小，胆管癌的发病率从2.7％到37.5％不等，总发病率为6.6％。关于术前诊断检查的详细报告很少，而是多模式检查，包括肝脏超声检查，计算机断层扫描,磁共振成像和内镜逆行胰胆管造影术用于大多数研究。疾病持续时间是可变的，长达几年。大多数病人有胆管炎发作,脓毒症,发烧或腹痛。在大多数情况下，肿瘤检测是手术标本的偶然发现，因为目前通常不可能在术前诊断期间检测到肿瘤表现。根据胆道病理的程度以及肝脏结构或功能的其他改变，进行了肝切除或肝移植。无术后辅助化疗报告，但对部分肿瘤复发病例给予化疗。在观察期间，一年后的总生存率为36％，复发率高达75％。
结论：只有少数回顾性研究报告肿瘤发病率较低。尽管死亡率和肿瘤复发率很高，应尽快提供明确的手术治疗。
