胰腺癌的不典型64排螺旋CT影像表现

目的 分析胰腺癌的64排螺旋CT的不典型表现,以提高对该肿瘤的CT征象的认识水平.方法 回顾性分析经手术病理证实的缺乏典型CT征象的12例胰腺导管腺癌的64排螺旋CT资料.结果 12例均为胰腺导管腺癌.其中,中分化导管腺癌7例,中高分化导管腺癌1例;黏液腺癌1例;腺鳞癌3例.8例导管腺癌病灶中位于胰头及(或)钩突部7例,胰颈部1例,表现为等、低密度或囊实性肿块,增强后无明显强化;5例肿瘤呈明显外生性或有外生倾向;5例肿瘤远端胰管无扩张,2例出现胆总管和肝内胆管扩张,仅1例出现肿瘤远端胰腺萎缩.1例黏液腺癌CT平扫示胰头部5 cm囊性病灶,增强后仅囊性病灶下方少许实性部分轻度强化,体尾部胰管中度扩张(7 mmn),胆总管及邻近血管未受侵犯.3例腺鳞癌病灶中位于胰头2例,胰体部1例,肿块最大径3.0～4.5 cm,CT增强扫描胰腺实质期示3例病灶内均见液化坏死区,病灶远端胰管均轻度扩张(4～5 mm),胆总管和肝内胆管均未见扩张.结论 胰腺癌可出现不典型的CT影像表现,要注意与易混淆疾病进行鉴别诊断.     

Solitary Cystic Dilation of the Intrahepatic Bile Duct: Morphology of Two Autopsy Cases and a Review of the Literature

Solitary cystic dilation of intrahepatic bile ducts with neither extrahepatic biliary anomalies nor renal lesions has been reported previously in only 11 cases. We report two cases which were found as a result of postmortem cholangiography of 149 livers at autopsy. Case 1 had a cystic dilation measuring 1.2 cm in diameter in the left lateral superior area duct. Case 2 showed a cystic dilation measuring 1.0 cm in diameter in the left lateral segment duct. Histologically, the walls of the dilated ducts consisted of fibrous wall with proliferation of periductal mucous glands in both cases. The dilated and adjacent bile ducts in case 2 also contained tiny brown pigment stones and biliary sludge, suggesting that this bile duct lesion is important in the formation of intrahepatic calculi by providing a site for bile stasis and mucous hypersecretion.     

Studies on cholangiographic and endoscopic findings of primary intrahepatic cholesterol stones

Cholangiographic investigations in 16 patients with primary intrahepatic cholesterol stones yielded some characteristic findings as follows. 1. Several numbers of stones were filled within locally dilated portion of the subsegmental or more peripheral intrahepatic bile duct branches, with no or minimal dilatation of the more peripheral portion and no stenosis-like lesions of the more hilar portion. 2. The lesion was single or multiple scattered in the liver and intrahepatic cholangiogram excluding involved segments was almost normal. 3. Although extrahepatic bile duct and/or gallbladder stones were often associated, the normal gallbladder, cystic duct and papillary portion and slightly dilated common bile duct were demonstrated. These cholangiographic findings were compatible with the endoscopic findings that inflammatory changes of the bile duct were rarely observed. According to above mentioned findings, it is suggested that the etiology of this disease differs from intrahepatic stones of calcium bilirubinate and dissolution therapy with bile acid may be available.     

Spontaneous necrosis of solid gallbladder adenocarcinoma accompanied with pancreaticobiliary maljunction

A 71-year-old Japanese man with acute cholecystitis and an incarcerated gallbladder （GB） stone was admitted. Plain ultrasonography （US） incidentally detected a mass-like lesion in the fundus. Doppler US revealed that this elevated lesion had no blood flow. Computed tomography showed a relatively low-density mass, measuring 5 cm x 4 cm in diameter, with no positive enhancement. Magnetic resonance imaging showed a mass in the fundus with a slightly low intensity on Tl-weighted images and a slightly high intensity on T2-weighted images. We were agonized in making the qualitative diagnosis of mass-like lesions of the fundus, such as a benign tumor, cancer, or debris. We performed laparoscopic cholecystectomy, because the incarcerated GB stone clearly caused acute cholecystitis. Intra-operative cholangiography clearly revealed pancreaticobiliary maljunction. Amylase levels in the common bile duct and gallbladder were quite high. The elevated lesion in the fundus dearly showed severe necrosis. Although this necrotic nodule included non-viable adenocarcinoma cells, viable cancer cell nests were located in the muscularis propria and subcutaneous layer. Histopathological examination confirmed a solid adenocarcinoma. Thus, we diagnosed it as a gallbladder cancer, based on histopathological analysis of the resected specimen. We therefore undertook radical surgery, including wedge resection of the liver, radical dissection of regional lymph nodes, and resection of the extrahepatic bile duct. Histopathological findings revealed no cancer, hyperplasia or dysplasia in the additionally resected specimens. The patient was finally staged as T2, N0, H0, P0, M（-）, stage Ⅱ. We present the first case of spontaneous necrosis of solid gallbladder adenocarcinoma, with a review of previous studies.     

Imaging diagnosis of 12 patients with hepatic tuberculosis

AIM: To assess CT, MR manifestations and their diagnostic value in hepatic tuberculosis. METHODS: CT findings in 12 cases and MR findings in 4 cases of hepatic tuberculosis proved by surgery or biopsywere retrospectively analyzed. RESULTS: (1) CT findings: One case of serohepatic type of hepatic tuberculosis had multiple-nodular lesions in the subcapsule of liver. Parenchymal type was found in 10 cases, including multiple, miliary, micronodular and low-density lesions with miliary calcifications in 2 cases; singular, low-density mass with multiple flecked calcifications in 3 cases; multiple cystic lesions in i case; multiple micronodular and low-density lesions fusing into multiloculated cystic mass or “cluser“ sign in 3 cases; and singular, macronodular and low-density lesion with multiple miliary calcifications in 1 case. One case of tuberculous cholangitis showed marked dilated intrahepatic ducts with multiple flecked calcifications in the porta hepatis. (2) MR findings in 4 cases were hypointense on both Tl-weighted imagings and T2-weighted imagings in one case, hypointense on Tl-weighted imagings and hyperintense on T2-weighted imagings in 3 cases. Enhanced MR in 3 cases was slightly shown peripheral enhancementor with multilocular enhancement. CONCLUSION: Various types of hepatic tuberculosis have different imaging findings, and typical CT and MR findingscan suggest the diagnosis.     

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.     

Intraductal papillary neoplasm of the bile duct developing in a patient with primary sclerosing cholangitis: A case report

We report a case of intraductal papillary neoplasm of the bile duct (IPNB) that developed in a patient with primary sclerosing cholangitis. A 46-year-old woman was admitted to our hospital with obstructive jaundice. The liver function tests demonstrated increased serum liver enzyme levels. Computed tomography showed dilatation of the intrahepatic bile ducts. Abdominal ultrasonography revealed a highly echoic protruding lesion in the posterior bile duct near the right lobe of the liver. The lesion was suspected to be IPNB, but we were unable to confirm whether it was a carcinoma. A right hepatectomy was performed, and this showed that the dilated bile duct was filled with mucin and contained several yellowish papillary tumors. Histologically, the neoplastic biliary epithelium showed papillary growth in the dilated lumen. The tumor was diagnosed as IPNB, high-grade intraepithelial neoplasia secreting abundant mucin. No recurrence has been detected 3 years after surgery.     

Intraductal papillary cholangiocarcinoma with aneurismal dilation: a case of the mimicking abscess

A case of cystic intraductal papillary cholangiocarcinoma is presented. A 58-year-old male patient presented with fever, chills, epigastric and right upper quadrant pain for 15 days. Clinically and radiologically, he was diagnosed as having liver abscess. After ultrasonography and abdominal computed tomography were taken, percutaneous drainage was performed and pus was drained. Computed tomography revealed a huge, multiloculated, septated cystic lesion in the right lobe of the liver. On the pathologic exam, the cystic lesion was an aneurismally dilated tumor, which spread diffusely and contiguously along the intrahepatic bile duct and microscopically the lesion was composed of a single layer of tall columnar tumor cells with short intraluminal papillary projections. We present the unusual case of intraductal papillary cholangiocarcinoma with aneurismal dilation and with superimposed infection.     

Intraductal papillary neoplasm of the bile duct extending superficially from the intrahepatic to extrahepatic bile duct

Intraductal papillary neoplasm of the bile duct (IPNB) or liver is a recently noted rare disease, and its pathogenesis remains unclear. Here we present a case of IPNB with an interesting morphology, which was treated by resection of the right hemiliver and extrahepatic bile duct. A 79-year-old woman was found to have a high alkaline phosphatase level and slight dilatation of the right intrahepatic bile duct on imaging studies. The right intrahepatic bile duct became dilated over a 2-year period; however, no solid mass could be detected, and tumor markers were not elevated. Hepatic resection was scheduled because a mucin-producing bile duct carcinoma of the liver was suspected. A right hemihepatectomy was conducted, and the extrahepatic bile duct was also resected after malignant cells were found in the surgical stump of the right bile duct and in the bile itself. Macroscopically, diffuse dilatation of the intrahepatic bile duct was noted, but no solid component or mucin within the duct was found. Histopathological findings revealed carcinoma in situ, IPNB, in the majority of intrahepatic bile ducts, with no lymph node metastasis, and it extended continuously to the epithelium of the common bile duct. No tumor recurrence or biliary dilatation was observed at follow-up 2 years after surgery. It is important to consider malignancy in the presence of a dilated bile duct and in the absence of any cause of occlusion. Complete resection of IPNB results in a good prognosis and no recurrence.     

自身免疫性胰腺炎的影像特征及其诊断价值

目的 探讨自身免疫性胰腺炎(AIP)的影像特征及其在AIP诊断中的价值.方法 回顾性分析13例AIP患者的影像和临床资料.结果 11例AIP表现为胰腺弥漫性肿大,2例胰头局限性肿大.CT平扫病变密度均均匀.4例在MR T1WI上信号降低、T2WI上信号轻度升高,增强后动脉期病变轻度强化,门脉期及延迟期进一步强化.9例胰腺周围有包膜样结构.横轴位图像上肝内外胆管扩张、胆总管胰腺段狭窄或闭塞10例,胰管未显影11例.6例行MRCP者有4例显示胆总管胰腺段较大范围狭窄或闭塞,胰管节段性狭窄.7例ERCP显示胰管弥漫性、不规则狭窄.胰周静脉受累8例,肾脏多发低密度灶6例,腹膜后纤维化2例,肝门部胆管狭窄1例,肺间质病变1例,强直性脊柱炎1例.结论 AIP的影像学征象具有一定特征性,影像检查在AIP诊断中起重要作用.识别胰腺外脏器受累对正确诊断AIP有帮助.     

Magnetic resonance imaging of cholangiocarcinoma

Cholangiocarcinoma arises from the bile ducts and is the most common primary malignancy of the biliary tree. Cholangiocarcinoma is classified according to its growth pattern: mass-forming, periductal-infiltrating, or intraductal-growing type. The majority of cholangiocarcinomas occur at the common hepatic duct (CHD) and its bifurcation, also referred to as Klatskin's tumor, but they also can occur in more peripheral branches within the hepatic parenchyma. Microscopically, cholangiocarcinoma represents an adenocarcinoma with a glandular appearance arising from the epithelium of the bile ducts. On magnetic resonance (MR) images, cholangiocarcinomas appear hypointense on T1-weighted images, and hyperintense on T2-weighted images. Central hypointensity can be seen on T2-weighted images and correspond to fibrosis. On dynamic MR images, cholangiocarcinomas show moderate peripheral enhancement followed by progressive and concentric filling in the tumor with contrast material. Pooling of contrast within the tumor on delayed MR images is suggestive of peripheral cholangiocarcinoma. The role of MR imaging in hilar cholangiocarcinoma is to confirm/reach a diagnosis and to assess resectability. Hilar cholangiocarcinoma shows the same signal intensity pattern of peripheral tumors both on T1- and T2-weighted images. On magnetic resonance cholangiopancreatography (MRCP) images, hilar cholangiocarcinoma appears as a moderately irregular thickening of the bile duct wall (>/=5 mm) with symmetric upstream dilation of the intrahepatic bile ducts. The aim of preoperative investigation in Klatskin tumors typically requires the evaluation of the level of biliary obstruction, the intrahepatic tumor spread, and the vascular involvement; it also needs to show any atrophy-hypertrophy complex. Because of its intrinsic high tissue contrast and multiplanar capability, MR imaging and MRCP are able to detect and preoperatively assess patients with cholangiocarcinoma, investigating all involved structures such as bile ducts, vessels and hepatic parenchyma. The main reason for surgical/imaging discrepancy is represented by the microscopic diffusion along the mucosa and in the perineural space.     

Pathogenesis and classification of intrahepatic cholangiocarcinoma: different characters of perihilar large duct type versus peripheral small duct type

Intrahepatic cholangiocarcinomas (ICCs) are made up of heterogenous carcinomas arising from different anatomical sites of the liver. Two types of candidate stem/progenitor cells of the biliary tree are postulated to exist at the peribiliary glands for large bile ducts and at the canals of Hering for small ducts and hepatocytes. According to the recent observations, ICCs can be subclassified into two types: tumors involving the large bile ducts comparable in size to the intrahepatic second branches and composed of a tubular or papillary component with tall columnar epithelium, and tumors involving the smaller duct than segmental branches and composed of small tubules with cuboidal epithelium. Perihilar large duct type ICCs can be interpreted as arising from large bile duct type ICCs, and peripheral small duct type ICCs may arise from small bile duct type or ductular type ICCs. Chronic biliary inflammation induces neoplastic change of the large bile ducts and thereby progression to the perihilar large duct type ICC, which can be grossly classified into periductal filtrating type ICC and intraductal growth type ICC, while chronic hepatitis or cirrhosis induces mass‐forming peripheral small duct type ICC. The different morphological and molecular features, including stromal components and tumor vasculature, support the hypothesis that perihilar large duct type ICCs and peripheral small duct type ICCs arise from different backgrounds, have different carcinogenetic pathways, and exhibit different biologic behaviors.     

Acute myeloid leukemia presenting as obstructive jaundice.

We report a 32-year-old man with acute myeloid leukemia presenting as obstructive jaundice. Imaging revealed dilated common bile duct with abrupt narrowing at the lower end, distended gall bladder, and dilated intrahepatic biliary radicles. In addition he had a mass lesion in the urinary bladder. On evaluation he was found to have the eosinophilic variant of M4 subtype acute myeloid leukemia. He expired before chemotherapy could be instituted.     

A case of asymptomatic intraductal papillary neoplasm of the bile duct without hepatolithiasis

A 65-year-old woman was found to have dilatation of the intrahepatic bile duct in the right anterior segment during a general health. Laboratory data were within normal ranges and no solid mass was detected in her abdominal computer tomography （CT） or nuclear magnetic resonance imaging （MRI）. However, endoscopic retrograde cholangiopancreatography （ERCP） demonstrated an obstruction of the right bile duct. Intraoperative cholangiography showed stenosis of the intrahepatic bile duct in the anterior inferior segment （B5） and narrowness of the intrahepatic bile duct in the anterior superior segment （B8）, so that we strongly suspected intrahepatic cholangiocarcinoma （ICC）. Histologically, surgically resected liver specimens, without tumor mass by macroscopic observation, showed intraductal papillary proliferation with fibrovascular cores and intraductal spreading of carcinoma in situ throughout a considerable area, especially in bile ductules around the peripheral small portal area. Furthermore, the immunohistochemical profile of the tumor （MUC5AC＋/CK7＋） was compatible with an intraductal papillary neoplasm of the bile duct （IPN-B）. Consequently, this case was diagnosed as IPN-B with spreading CIS, stageⅠ（pT1, pN0, P0, H1, M0）. We report a case of IPN-B with interesting histopathologicalfindings and emphasize that cholangiography is especially helpful for the diagnosis of bile duct dilatation due to infiltration of carcinoma cells.     

Multicystic biliary hamartoma with extremely elevated CA19-9: a case report

Multicystic biliary hamartoma (MCBH) is a rare cystic disease of the liver. We herein report a case of MCBH associated with extremely elevated levels of serum carbohydrate antigen (CA) 19-9. A 53-year-old man was referred to our hospital because of extremely elevated CA19-9 levels (more than 12,000?U/mL). Enhanced abdominal computed tomography and magnetic resonance imaging (MRI) revealed a multicystic tumor with a calcified wall in the left lobe of the liver, although no apparent intracystic nodule was detected. Because of the possibility of a malignant tumor, such as intraductal papillary neoplasm of the bile duct or cystadenocarcinoma, the patient underwent left hepatectomy. Based on the postoperative pathological findings, the lesion was diagnosed as MCBH. The serum CA19-9 level drastically decreased after surgery. We encountered a rare case of MCBH with extremely elevated CA19-9 levels.     

Diffuse intrahepatic bile duct dilation caused by a very small hepatic cyst

A 72-year-old woman presented to our hospital with diffuse dilation of the intrahepatic bile ducts. Imaging studies revealed a solitary hepatic cyst, 3?cm in diameter, in segment 4 of Couinaud's category of the liver, riding on the hilar hepatic duct. Deroofing of the hepatic cyst was performed, and the dilation of the intrahepatic bile ducts was attenuated. We concluded that even a very small solitary hepatic cyst might cause stenosis of the common hepatic duct, if it is located just above the hepatic hilum.     

Experimental liver cysts induced by 2-acetylaminofluorene

Liver cyst formation was studied serially in an experimental model in which rats were fed a diet containing 0.02% 2-acetylaminofluorene, a carcinogen, for 6 weeks, followed by a normal diet for 42 weeks. Cysts appeared in the portal tracts at the 12th week, by which time the intrahepatic bile ducts had also proliferated and dilated. Some of the dilated bile ducts were cavernous or multicystic and appeared to represent a transitional form between the bile duct dilation and cystic formation. After 20 weeks cysts were observed in the majority of rats. The number and the size of cysts in the liver increased with time; after 40 weeks, there were up to 20 cysts as large as 15 mm in diameter. Carcinoma or cirrhosis did not develop in this model. Indocyanine green dye injected intravenously did not accumulate in the cysts, which denies the possibility of communication between cysts and the bile duct. Scanning electron microscopy demonstrated that the cysts were composed of a few intercommunicating cavities and were lined with epithelial cells similar to those of the intrahepatic bile duct, which had numerous microvilli and a central cilium. These observations suggest that liver cysts originate in the bile duct.     

Living-donor liver transplantation for Caroli's disease with intrahepatic adenocarcinoma

A 36-year-old woman who had Caroli's disease with refractory cholangitis and complicated intrahepatic cholangiocarcinoma was successfully treated with living-donor liver transplantation. Preoperative computed tomography and ultrasonography showed a small nodule in the dilated intrahepatic bile duct. In the resected liver specimen, a small papillary tumor was located in the dilated intrahepatic bile duct of the right lobe. The pathological finding revealed a well differentiated papillary adenocarcinoma without invasion to the parenchyma. The patient is currently doing well 2.5 years after transplantation, with no signs of recurrence of the disease. For Caroli's disease, we believe we can achieve good results with liver transplantation, not only for cholangitis but also for the carcinoma when it is localized in the liver and the patient is carefully followed up.     

Pathological classification of intrahepatic cholangiocarcinoma based on a new concept

Intrahepatic cholangiocarcinoma (ICC) arises from the lining epithelium and peribiliary glands of the intrahepatic biliary tree and shows variable cholangiocytic differentiation. To date, ICC was largely classified into adenocarcinoma and rare variants. Herein, we propose to subclassify the former, based on recent progress in the study of ICC including the gross classification and hepatic progenitor/stem cells and on the pathological similarities between biliary and pancreatic neoplasms. That is, ICC is classifiable into the conventional (bile duct) type, the bile ductular type, the intraductal neoplasm type and rare variants. The conventional type is further divided into the small duct type (peripheral type) and large bile duct type (perihilar type). The former is a tubular or micropapillary adenocarcinoma while the latter involves the intrahepatic large bile duct. Bile ductular type resembles proliferated bile ductules and shows a replacing growth of the hepatic parenchyma. Hepatic progenitor cell or stem cell phenotypes such as neural cell adhesion molecule expression are frequently expressed in the bile ductular type. Intraductal type includes papillary and tubular neoplasms of the bile duct (IPNBs and ITNBs) and a superficial spreading type. IPNB and ITNB show a spectrum from a preneoplastic borderline lesion to carcinoma and may have pancreatic counterparts. At invasive sites, IPNB is associated with the conventional bile duct ICC and mucinous carcinoma. Biliary mucinous cystic neoplasm with ovarian-like stroma in its wall is different from IPNB, particularly IPNB showing cystic dilatation of the affected ducts. Rare variants of ICC include squamous/adenosquamous cell carcinoma, mucinous/signet ring cell carcinoma, clear cell type, undifferentiated type, neuroendocrine carcinoma and so on. This classification of ICC may open up a new field of research of ICC and contribute to the clinical approach to ICC.