细胆管癌的临床病理特征

细胆管癌(cholangiolocellular carcinoma, CLC)由于其特殊的细胞起源,具有肝细胞癌和胆管细胞癌的双重临床病理和影像学特点,由于惰性生长的特点、预后相对较好,其生长特点和临床特征明显有别于传统的肝内胆管癌(intrahepatic cholangiocarcinoma,ICC),是一种特殊类型的原发性肝脏恶性肿瘤.在细胞起源、临床病理特征、生长特点和预后等方面与传统意义的ICC是两种截然不同的疾病,但国内外有关ICC的研究中往往将两者混为一谈,没有明确的区分.本文就CLC临床病理特征进行综述,以期引发大家对这一问题的重视,并加强相关的研究.     

182例肝内胆管细胞癌临床病理分析

肝内胆管癌(intrahepatic cholangiocarcinoma,ICC)是起源于肝内二级胆管以下的胆管细胞恶性肿瘤,较为少见,在病因、临床表现、治疗方法和预后方面均与肝细胞癌及肝外胆管细胞癌等有较大差异.现回顾性分析我院182例ICC患者的临床资料,探讨其病因和临床特点.     

抗-HBs、抗-HBe和抗-HBc与肝内胆管细胞癌的相关性

肝内胆管细胞癌（ICC）是来源于肝内胆管上皮细胞的恶性肿瘤,约占原发性肝癌的5％。以往的研究资料提示该疾病的发生可能与HBV或HCV感染、肝内胆管结石、原发性硬化性胆管炎（PSC）、肝吸虫感染等有关。最近研究提示,曾经感染过HBV经自身清除后血清中出现抗-HBs和（或）抗-HBc阳性,或HBV隐匿性感染（血清中HBsAg阴性,但是血清或肝组织中HBV DNA持续存在）是发生肝细胞癌（HCC）的高危因素,与肝内胆管细胞癌是否有相关性,尚无相关报道。     

原发性肝癌诊疗指南(2022年版)国家卫生健康委办公厅

1概述原发性肝癌是目前我国第4位常见恶性肿瘤及第2位肿瘤致死病因,严重威胁我国人民的生命和健康[1-3]。原发性肝癌主要包括肝细胞癌(hepatocellular carcinoma,HCC)、肝内胆管癌(intrahepatic cholangiocarcinoma,ICC)和混合型肝细胞癌-胆管癌(combined hepatocellular-cholangiocarcinoma,cHCC-CCA)三种不同病理学类型,三者在发病机制、生物学行为、病理组织学、治疗方法以及预后等方面差异较大,其中HCC占75%~85%、ICC占10%~15%[4]。     

趋化因子/受体轴调控肝癌免疫细胞招募及转移的研究进展

<正>肝癌是一种常见的消化系统恶性肿瘤,主要包括三种不同的病理类型,分别为肝细胞癌(HCC)、肝内胆管细胞癌(ICC)和混合性肝细胞和胆管细胞癌(HCC-ICC)。在所有原发性肝癌中,HCC是最常见的类型,占所有原发性肝癌的85%～90%,其次是ICC,占所有原发性肝癌的15%^[1]。2020年的一项研究所示,在全球范围内,每年肝癌的新发病例数在所有恶性肿瘤中排名第7,在癌症相关死亡病例中排名第3^[2]。肝癌的早期往往没有明显症状,     

原发性肝癌诊疗指南(2022年版)

原发性肝癌是目前我国第4位常见恶性肿瘤及第2位肿瘤致死病因, 严重威胁我国人民的生命和健康[1-3]。原发性肝癌主要包括肝细胞癌(hepatocellular carcinoma, HCC)、肝内胆管癌(intrahepatic cholangiocarcinoma, ICC)和混合型肝细胞癌-胆管癌(combined hepatocellular-cholangiocarcinoma, cHCC-CCA)3种不同病理学类型, 三者在发病机制、生物学行为、病理组织学、治疗方法以及预后等方面差异较大, 其中HCC占75%～85%、ICC占10%～15%[4]。本指南中的"肝癌"仅指HCC。     

超声内镜诊断肿块型肝内胆管细胞癌1例

<正>肝内胆管细胞癌(intrahepatic cholangiocarcinoma, ICC)又称肝内胆管癌,是起源于肝内2级胆管及其分支上皮的恶性肿瘤,好发于肝左叶,占胆管细胞癌的20%～25%[1],是发病率居第2的肝脏原发恶性肿瘤,近年发病率呈上升趋势,早期缺乏特异的临床症状、血清学检查和早期筛查的分子标志物,诊断较困难。超声内镜(endoscopic ultrasonography, EUS)以及衍生技术在“早期”及“精准”的道路上上不断改革更新,已有国内外文献报道其在诊断及鉴别诊断肝脏疾病上有明显效果。在此,     

趋化因子在肝癌中的作用

<正>原发性肝癌(primary hepatocellular carcinoma,PHC)主要包括肝细胞癌(hepatocellular carcinoma,HCC)、肝内胆管细胞癌(intrahepatic cholangiocarcinoma ICC)和肝细胞癌-肝内胆管细胞癌混合型等不同病理类型,其发病机制、生物学行为、组织学形态、临床表现、治疗方法以及预后等方面均有明显的不同[1];由于其中HCC占到90%以上,故本文所指的"肝癌"主     

超声造影对不同病理类型及分化程度原发性肝癌的诊断价值

原发性肝癌(PLC)为最常见的肝局灶性病变之一,肝细胞癌(HCC)与肝内胆管细胞癌(ICC)为PLC的不同病理类型,明确诊断对临床治疗及评估预后具有重要意义.本研究对不同病理组织来源及分化程度PLC的超声造影(CEUS)特点结合定量参数进行回顾性分析,探讨CEUS对PLC诊断及鉴别诊断的价值.     

靶向Hippo信号通路治疗原发性肝癌的研究进展

<正>原发性肝癌(肝癌)是临床常见恶性肿瘤,包括肝细胞癌(HCC)和肝内胆管细胞癌(ICC)。近年来,肝癌发病率呈不断上升趋势,2020年全球原发性肝癌发病率为4.7%,死亡率为8.3%,位居全球常见恶性肿瘤第5位和肿瘤致死病因第3位^[1]。因早期诊断难,且具有高转移、高复发等特点,大多数患者确诊时已处于中晚期,导致肝癌预后差,5年生存率低于15%^[2]。     

Intrahepatic cholangiocellular carcinoma and hepatocellular carcinoma developed after a 6-year sustained virological response to interferon therapy for chronic hepatitis C

A 67-year-old male patient presenting with chronic hepatitis C (CHC) achieved a sustained virological response (SVR) following 6 months of treatment with 6 million units of beta-interferon (IFN). The SVR state continued for 6 years. Hepatocellular carcinoma (HCC) developed in liver segments 4 and 5, and was treated with transcatheter arterial chemoembolization, followed by radiofrequency ablation of the tumors. A recurrence of HCC occurred in segment 4 one and a half years after the initial treatment for HCC and a new tumor also developed in segment 8. These tumors were diagnosed to be recurrent HCC, and the three hepatic segments were resected. The pathological examination and immunostaining of the tumors revealed the tumor in segment 4 to be a well to moderately differentiated typical HCC. On the other hand, the tumor in segment 8 was a moderately to poorly differentiated adenocarcinoma and was diagnosed as an intrahepatic cholangiocellular carcinoma (ICC). HCC developed from CHC in a patient who achieved a 6-year SVR after IFN therapy, followed one and a half years later by the development of a heterochronous ICC at a different site, thus indicating the presence of HCC–ICC double cancer. This was an exceedingly rare and clinically important case in terms of the carcinogenic mechanism of HCC and ICC from a post-SVR CHC patient. We have to be aware of the possible development not only of HCC but of ICC after SVR in CHC patients.     

A case of cholangiolocellular carcinoma asynchronously developing 3 years after the resection of hepatocellular carcinoma

Cholangiolocellular carcinoma (CLC) is a rare malignant primary liver tumor, categorized as a subtype of cholangiocellular carcinoma. CLC is considered to originate from hepatic progenitor cells, and sometimes accompanies a component of cholangiocellular carcinoma and/or hepatocellular carcinoma (HCC). We report herein a case of a 53-year-old Japanese male with CLC occurring asynchronously after a resection of HCC. At the age of 50, the patient underwent a first hepatectomy due to HCC, followed by intrahepatic recurrence. At 53, a new type of hepatic tumor was detected. Dynamic computed tomography revealed a multinodular enhanced tumor in the hepatic arterial phase, and an iso-density tumor in the portal venous and delayed phases. Living donor liver transplantation was performed for the treatment of this liver tumor because of his poor liver function. Histological findings confirmed the diagnosis of CLC. Nineteen months after the liver transplantation, bone metastasis of CLC was detected. Although the preoperative diagnosis of CLC is difficult in general, CLC should be considered as a differential diagnosis when a tumor with atypical image findings for HCC is observed after the resection of HCC. Once the diagnosis of CLC is made, postoperative surveillance of the patient must be performed, including the bone.     

Precancerous lesions of intrahepatic cholangiocarcinoma

Intrahepatic cholangiocarcinoma (ICC), also known as cholangiocellular carcinoma or peripheral bile duct carcinoma, is an intrahepatic malignant tumor that consists of cells resembling the biliary epithelium. The proportion of ICC among primary hepatic malignancies is approximately 10% worldwide. Although the etiology and pathogenesis remain unclear in a great majority of cases, preceding pathologic conditions or etiologies in the development of ICC are known or suspected in a proportion of these patients. Some forms of ICC, such as those associated with hepatolithiasis or liver fluke infestation, are endemic in parts of the world, particularly in East Asia. ICC is reportedly a late complication of primary sclerosing cholangitis. Most of these preceding pathologic conditions are forms of chronic cholangitis, and longstanding inflammation, chronic injury, and regenerative hyperplasia of the biliary epithelium may be causally related to malignant transformation. Biliary epithelial dysplasia is encountered in the intrahepatic bile ducts both near and remote from ICC foci in the liver and near and remote from the chronically inflamed biliary tree. This lesion could be a precancerous lesion, and it shows telomerase activity and increased proliferative activities. Furthermore, congenital and developmental disorders of the biliary system, such as Caroli's disease, congenital hepatic fibrosis, and pancreatico-biliary malformation are occasionally associated with ICC. Benign biliary tumors, such as biliary papillomatosis, may eventually undergo malignant transformation. These lesions could be regarded as precancerous or borderline lesions. Some ICCs may also develop in nonbiliary viral cirrhosis. In the majority of cases of ICC, however, the etiology, pathogenesis, and developmental processes and precancerous lesions of the ICC remain unclear.     

746例肝脏和肝内胆管肿瘤临床发病特点分析

目的探讨肝脏和肝内胆管肿瘤的临床发病特点。方法收集2003年~2011年我科诊断的肝脏和肝内胆管肿瘤746例,分析其发病特点。结果肝脏肿瘤发病位于前九位的依次为肝细胞癌（43.4%）、血管瘤（37.3%）、肝内胆管细胞癌（5.8%）、转移性肿瘤（3.8%）、造血和淋巴样肿瘤（1.1%）、胆道乳头状瘤病（0.94%）、局灶性结节状增生（0.94%）、肝细胞不典型增生（0.67%）和不典型增生结节（0.54%）;本组未发现孤立性纤维肿瘤、淋巴管瘤病、假脂肪瘤、胚胎性肉瘤、Kaposi肉瘤、上皮样血管内皮细胞瘤、血管肉瘤、癌肉瘤、横纹肌肉瘤、畸胎瘤、卵黄囊瘤和横纹肌样瘤;肝细胞癌的发病年龄高峰在40~69岁,男性比女性高发,肝内胆管细胞癌高发年龄为50~69岁,男性与女性发病人数相当;血管瘤发病年龄高峰为30~49岁,女性比男性高发;转移性肿瘤发病高峰为40~59岁,男性与女性发病人数相当。结论肝脏肿瘤发病有逐年递增的趋势,来源于上皮的肝细胞癌是肝脏最常见的恶性肿瘤,来源于间叶的血管瘤是最常见的良性肿瘤。     

Colonic metastasis of Klatskin tumor： Case report and discussion of the current literature

INTRODUCTIONBile duct tumors are rare neoplasms with an incidence of 0.5-1/100 000[1]. Predisposing factors include mainly PSC with a thirty-fold increased risk as well as choledochal cysts and parasitic infections (clonarchis sinensis, opisthordis viverr…     

Hepatocellular carcinoma with obstructive jaundice： diagnosis,treatment and prognosis

Obstructive jaundice as the main clinical feature is uncommon in patients with hepatocellular carcinoma (HCC). Only 1-12 % of HCC patients manifest obstructive jaundice as the initial complaint. Such cases are clinically classified as “icteric type hepatoma”, or “cholestatic type of HCC”. Identification of this group of patients is important, because surgical treatment may be beneficial. HCC may involve the biliary tract in several different ways: tumor thrombosis, hemobilia,tumor compression, and diffuse tumor infiltration. Bile duct thrombosis (BDT) is one of the main causes for obstructive jaundice, and the previously reported incidence is 1.2-9 %.BDT might be benign, malignant, or a combination of both.Benign thrombi could be blood clots, pus, or sludge.Malignant thrombi could be primary intrabiliary malignant tumors, HCC with invasion to bile ducts, or metastatic cancer with bile duct invasion. The common clinical features of this type of HCC include: high level of serum AFP, history of cholangitis with dilation of intrahepatic bile duct; aggravating jaundice and rapidly developing into liver dysfunction. It is usually difficult to make diagnosis before operation, because of the low incidence rate, ignorant of this disease, and the difficulty for the imaging diagnosis to find the BDT preoperatively. Despite recent remarkable improvements in the imaging tools for diagnosis of HCC, such cases are still incorrecty diagnosed as cholangiocarcinoma or choledocholithiases. Ultrasonography (US) and CT are help fulin showing hepatic tumors and dilated intrahepatic and/or extrahepatic ducts containing dense material correspondingto tumor diebris. Direct cholangiography including percutaneous transhepatic cholangiography (PTC) and endoscopic retrograde cholangiopancreatography (ERCP)remains the standard procedure to delineate the presence and level of biliary obstruction. Magnetic resonance cholangiopancreatography (MRCP) is superior to ERCP in interpreting the cause and depicting the anatomical extent of the perihilar obstructive jaundice, and is particularly distinctive in cases associated with tight biliary stenosis and along segmental biliary stricture. Choledochoscopy and bile duct brushing cytology could be alternative useful techniques in the differentiating obstructions due to intraluminal mass,infiltrating ductal lesions or extrinsic mass compression applicable before and after duct exploration. Jaundice is not necessarily a contraindication for surgery. Most patients will have satisfactory palliation and occasional cure if appropriate procedures are selected and carried out safely, which can result in long-term resolution of symptoms and occasional long-term survival. However, the prognosis of icteric type HCC is generally dismal, but is better than those HCC patients who have jaundice caused by hepatic insufficiency.     

Hemobilia immediately after transcatheter arterial chemoembolization using drug‐eluting beads for hepatocellular carcinoma with intrahepatic bile duct invasion

Transcatheter arterial chemoembolization (TACE) is used as a palliative treatment for unresectable hepatocellular carcinoma (HCC) worldwide. Recently, a novel drug delivery–embolic agent, the drug‐eluting bead (DEB), was introduced for TACE. There are a few reports of tumor hemorrhage after TACE using DEB (DEB‐TACE) for HCC. However, there have not been any reports of hemobilia immediately after DEB‐TACE for HCC with intrahepatic bile duct invasion. Here, the first such case is reported. A 71‐year‐old woman was admitted to our hospital to undergo DEB‐TACE for multiple HCCs with worsening left intrahepatic bile duct dilatation. She was diagnosed with HCC that extensively invaded the left hepatic duct. After DEB‐TACE through the left hepatic artery, a hepatic arteriogram showed extra flow of the contrast agent to the left hepatic and common bile ducts. Therefore, transcatheter arterial embolization (TAE) of the responsible vessel was carried out using coils, and no extra flow of the contrast agent was identified. The patient was discharged 14 days after TAE without deterioration of liver function. Although hemobilia immediately after DEB‐TACE is rare, there may be increased potential for hemobilia when DEB‐TACE is carried out for HCC with extensive bile duct invasion. We suggest that DEB‐TACE may be contraindicated for such cases.     

Synchronously resected double primary hepatic cancers — hepatocellular carcinoma and cholangiolocellular carcinoma

The frequency of double primary cancers in the liver is very low. All reported cases are double cancers consisting of hepatocellular carcinoma (HCC) and intrahepatic cholangiocellular carcinoma (CCC). We herein report a surgical patient who had simultaneous double cancers consisting of HCC and cholangiolocellular carcinoma (CoCC). This is the first case report of such a patient. A 70‐year‐old Japanese man was admitted to our hospital for further examination of two hepatic nodules. He had a history of schistosomiasis japonica, idiopathic pulmonary fibrosis, and diabetes mellitus. Laboratory data revealed that hepatitis C virus (HCV) antibody was positive and hepatic enzymes were slightly elevated. The level of prothrombin induced by vitamin K absence or antagonist II was elevated. Computed tomography depicted two tumors; one, measuring 4.0 cm in diameter, was in the medial segment and the other, 2.2 cm in diameter, was in the posterior superior segment of the liver. The larger tumor showed contrast enhancement and the smaller one showed enhancement at the tumor periphery in the hepatic arterial phase. In the portal phase, the larger tumor became less dense than the liver parenchyma, but the periphery of the smaller one showed continuous enhancement. He underwent an operation under a diagnosis of double hepatic cancers, consisting of HCC and CCC. However, microscopic examination of the resected tumors revealed that the larger tumor was moderately differentiated HCC and the smaller one was CoCC.     

肝门部汇管区胆管癌的超声表现

目的探讨肝门部汇管区（左肝管、右肝管与肝总管汇合部）胆管癌的超声诊断价值。方法常规肝胆超声探查,配合患者呼吸动作,在肝门区详细观察肝门部脉管,胆管的走行及分布状,肿块大小及边界,肝内胆管扩张程度和分布范围,远段肝外胆管显示状态,所伴行的门静脉及肝门部其它组织结构回声情况等。将异常所见照片记录,并与CT、MRI及手术结果对照分析。结果超声诊断54例汇管区胆管癌病例中,CT、MRI证实29例,手术证实25例,所有病例均有不同程度的肝内胆管扩张。结论汇管区胆管癌的直接、间接超声表现具有一定的特征性,超声对汇管区胆管癌的判定具有较高的诊断价值。     

A case of concomitant colitic cancer and intrahepatic cholangiocarcinoma during follow-up for ulcerative colitis

Colitis-associated colorectal cancer (CAC) is known to occur in long-standing and extensive ulcerative colitis (UC). Furthermore, UC is known to complicate primary sclerosing cholangitis (PSC), which subsequently results in an increased risk of developing cholangiocarcinoma. We report a case of colitis-associated rectal cancer (CARC) accompanied by intrahepatic cholangiocarcinoma (ICC) based on UC and PSC. A 73-year-old man had suffered from UC for 19 years. During surveillance colonoscopy, a tumor was found in the rectum that was pathologically diagnosed as CARC from the resected specimen. Abdominal computed tomography also revealed a localized dilation of the intrahepatic bile duct, and endoscopic retrograde cholangiography revealed a band-like stricture. This remarkable tumor lesion was not observed in the hepatic duct. Left hepatectomy was performed because of the suspicion of possible ICC at the stenosis of the hepatic duct. The presence of ICC was confirmed at the lesion causing the stricture. The pathological diagnosis from the resected specimen was ICC based on PSC. Adjuvant chemotherapy for ICC was performed for 6 months. Neither cancer has recurred for 2.5 years after hepatectomy. Patients with PSC concomitant with UC should be considered a high-risk group for CAC and ICC.