Excerpt from the 2022 American Association for the Study of Liver Diseases clinical practice guideline: management of primary sclerosing cholangitis and cholangiocarcinoma / 中华肝脏病杂志

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define "related stricture" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.

Current status and future perspectives on the methods of prognosis evaluation for intrahepatic cholangiocarcinoma / 中华外科杂志

Intrahepatic cholangiocarcinoma (ICC) is the second most common primary malignant tumor in the liver after hepatocellular carcinoma. Its incidence and mortality rates have increased worldwide in recent years. Surgical resection is the best treatment modality for ICC;however,the overall prognosis remains poor. Accurate evaluation of post operative prognosis allows personalized treatment and improved long-term outcomes of ICC. The American Joint Commission on Cancer TNM staging manual is the basis for the standardized diagnosis and treatment of ICC;however,the contents of stage T and stage N need to be improved. The nomogram model or scoring system established in the analysis of commonly used clinicopathological parameters can provide individualized prognostic evaluation and improve prediction accuracy;however,more studies are needed to validate the results before clinical use. Meanwhile,imaging features exhibit great potential to establish the post operative prognosis evaluation system for ICC. Molecular-based classification provides an accurate guarantee for prognostic assessment as well as selection of populations that are sensitive to targeted therapy or immunotherapy. Therefore,the establishment of a prognosis evaluation system,based on clinical and pathological characteristics and centered on the combination of multidisciplinary and multi-omics,will be conducive to improving the long-term outcomes of ICC after surgical resection in the context of big medical data.

Colangiocarcinoma y hepatocolangiocarcinoma combinado en pacientes con cirrosis / Cholangiocarcinoma and combined hepatocellular-cholangiocarcinoma in cirrhotic patients

Background: Cholangiocarcinoma (CCA) is a primary hepatic tumor, frequently found in patients with liver cirrhosis and biliary tract diseases. Its varieties include isolated CCA or "combined hepatocellular-cholangiocarcinoma" (cHCC-CCA). The latter is uncommon, with poorly defined diagnostic criteria and natural history. Aim: To characterize patients with cirrhosis with a pathological diagnosis of CCA and cHCC-CCA. Material and Methods: Forty-nine liver biopsies with a pathological diagnosis of CCA were reviewed. The clinical records of patients were reviewed to fetch demographic variables, etiology of cirrhosis and clinical presentation. Results: Eight of the 49 patients had cirrhosis (16% of CCA biopsies reviewed). Their median age was 64 (27-71) years and five were females. Four patients had CCA, three patients cHCC-CCA and one had a bifocal tumor. Patients in the CCA group were more commonly symptomatic. Alpha-fetoprotein and CA 19-9 levels were elevated in one of eight and four of six patients, respectively. Within 12 months from diagnosis, five of eight patients died. Conclusions: In most of these cases, the diagnosis of cHCC-CCA and CCA was made in the liver explant study without previous imaging diagnosis. This reinforces the usefulness of the histological study, in specific cases, prior to liver transplantation and emphasizes the importance of systematic explant exploration in these cases.

A novel nomogram for individualized preoperative prediction of lymph node metastasis in patients with intrahepatic cholangiocarcinoma / 中华外科杂志

Objective: Constructing and validating a nomogram model for preoperative prediction of intrahepatic cholangiocarcinoma (ICC) lymph node metastasis to assist decision making during surgery. Methods: Retrospectively collecting the clinical and pathological data of 1 031 ICC patients who underwent partial hepatectomy at Eastern Hepatobiliary Surgery Hospital of Naval Military Medical University,General Hospital of Eastern Theater Command,or Zhongda Hospital Southeast University from January 2003 to January 2014. There were 682 males and 349 females; mean age was 54.7 years(range:18 to 82 years). There were 562 patients who underwent lymph node dissection and 469 patients who did not. Among the patients in the dissection group,Lasso regression method was used to filtrate preoperative variables related to lymph node metastasis and establish a nomogram. Bootstrap method was used to internally validate the discrimination of the nomogram,and the accuracy of the nomogram was assessed by using calibration curves. Patients were divided into low-moderate and high-risk groups based on model prediction probability. Propensity score matching(PSM) was used to analyze the overall survival (OS) and recurrence-free survival (RFS) of patients with and without lymph node dissection in the two groups,and to judge the importance of lymph node dissection in the two groups. Results: Six factors related to ICC lymph node metastasis were determined by Lasso regression,including hepatitis B surface antigen,CA19-9,age,lymphadenopathy,carcinoembryo antigen and maximum tumor diameter. These factors were integrated into a nomogram to predict ICC lymph node metastasis. The aera under curve value was 0.764,and the C-index was 0.754. Stratified analysis showed that OS and RFS in the high-risk group of lymph node metastasis were significantly lower than those in the low-medium risk group(median OS:14.6 months vs. 27.0 months,P<0.01; median RFS:9.1 months vs. 15.5 months,P<0.01). In the high-risk group,the median OS was 16.7 months and 6.3 months(Log-rank test: P=0.187;Wilcoxon test:P=0.046),and the median RFS was 11.0 months and 4.8 months(P=0.403),respectively in the lymph node dissection group and undissected group after PSM. In the low-medium-risk group,the median OS was 22.7 months and 26.7 months(P=0.288),and the median RFS was 13.0 months and 14.5 months(P=0.306),respectively in the lymph node dissection group and undissected group after PSM. Conclusions: The nomogram could be used for preoperative prediction of lymph node metastasis and prognostic stratification in patients with ICC. For patients with high risk of lymph node metastasis predicted by the model,active dissection should be performed. For patients predicted to be at low-moderate risk,lymph node dissection might be optional in some specific cases.

Impact of adjuvant chemotherapy on prognosis in intrahepatic cholangiocarcinoma patients underwent radical resection / 中华外科杂志

Objectives: To investigate the clinical value of adjuvant chemotherapy(ACT) in patients with intrahepatic cholangiocarcinoma(ICC) who underwent radical resection and to explore the optimal population that can benefit from ACT. Methods: A retrospective cohort study method was adopted. The clinical and pathological data of 685 patients with ICC who underwent curative intent resection in 10 Chinese hepatobiliary surgery centers from January 2010 to December 2018 were collected;There were 355 males and 330 females. The age(M(IQR)) was 58(14) years (range: 22 to 83 years). Propensity score matching(PSM) was applied to balance the differences between the adjuvant and non-adjuvant chemotherapy groups. Log-rank test was used to compare the prognosis of the two groups of patients. A Bayesian network recurrence-free survival(RFS) prediction model was constructed using the median RFS time (14 months) as the target variable, and the importance of the relevant prognostic factors was ranked according to the multistate Birnbaum importance calculation. A survival prognostic prediction table was established to analyze the population benefiting from adjuvant chemotherapy. Results: Among 685 patients,214 received ACT and 471 did not receive ACT. A total of 124 pairs of patients were included after PSM, and patients in the ACT group had better overall survival (OS) and RFS than those in the non-ACT group(OS: 32.2 months vs. 18.0 months,P=0.003;RFS:18.0 months vs. 10.0 months,P=0.001). The area under the curve of the Bayesian network RFS prediction model was 0.7124. The results of the prognostic factors in order of importance were microvascular invasion (0.158 2),perineural invasion (0.158 2),N stage (0.155 8),T stage (0.120 9), hepatic envelope invasion (0.090 3),adjuvant chemotherapy (0.072 1), tumor location (0.057 5), age (0.042 3), pathological differentiation (0.034 0), sex (0.029 3), alpha-fetoprotein (0.028 9) and preoperative jaundice (0.008 5). A survival prediction table based on the variables with importance greater than 0.1 (microvascular invasion,perineural invasion,N stage,T staging) and ACT showed that all patients benefited from ACT (increase in the probability of RFS≥14 months from 2.21% to 7.68%), with a more significant increase in the probability of RFS≥14 months after ACT in early-stage patients. Conclusion: ACT after radical resection in patients with ICC significantly prolongs the OS and RFS of patients, and the benefit of ACT is greater in early patients.

Thinking and suggestion on the definition, classification and Chinese nomenclature of carcinoma of the bile ducts / 中华外科杂志

At present, the classification, nomenclature, and definition of carcinoma of the bile ducts are controversial. Moreover, there is no uniformity between China and aboard, which has brought confusion to clinical practice. It needs to clarify regarding tumor naming principles, anatomical location, tumor origin, pathological classification, biological characteristics, clinical manifestations, treatment methods, etc. Additionally, the WHO tumor classification, UICC staging, ICD disease classification, relevant Chinese regulations, EASL, AJCC staging, and NCCN guidelines were also needed to be referred. After investigating the above-mentioned latest authoritative literature, based on the existing problems, combined with clinical practice in China, the author reevaluated the definition, classification, and nomenclature of cholangiocarcinoma, and proposes updated suggestions. Hoping to standardize and unify clinical practice for classification and nomenclature of cholangiocarcinoma in China.

Value of albumin RNAscope in situ hybridization in diagnosis and differential diagnosis of hepatocellular carcinoma / 中华病理学杂志

Objective: To investigate the utility of albumin RNAscope in situ hybridization in the diagnosis and differential diagnosis of hepatocellular carcinoma and its mimics. Methods: One hundred and fifty-two cases of hepatocellular carcinoma and its mimics and 33 cases of normal tissue were selected from the pathology database of the Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from January 2013 to December 2019. Tissue microarrays were constructed and RNAscope in situ hybridization was performed to detect the expression of albumin mRNA. Results: No albumin mRNA expression was detected in normal tissues except for the liver. All hepatocellular carcinoma regardless of its degree of differentiation and primary or metastatic nature had detectable albumin mRNA, with strong and diffuse staining in 90.7% (49/54) of cases. While the positive rate of HepPar-1, Arg-1 or one of them by immunohistochemistry was 87.0% (47/54), 85.2% (46/54) and 92.6% (50/54) respectively. The positive rates of albumin mRNA in intrahepatic cholangiocarcinoma and biphenotypic hepatocellular carcinoma were 7/15 and 9/10, respectively. The former showed focal or heterogeneous staining, while the latter showed strong and diffuse staining. The positive rate of hepatoid adenocarcinoma was 8/19, and the albumin expression could be diffuse or focal. Sporadic cases of poorly differentiated gastric adenocarcinoma and metastatic colon adenocarcinoma showed focal staining of albumin mRNA. Conclusions: Detection of albumin mRNA by RNAscope in situ hybridization is of great value for the diagnosis and differential diagnosis of HCC, and the sensitivity may be improved by combining with HepPar-1 and Arg-1. It also offers different diagnostic clues according to different expression patterns.

Cavernoma portal complicada con biliopatía secundario a enfermedad de Gaucher tipo 1: reporte de un caso peruano / Cavernoma complicated with biliopatia secondary to type 1 Gaucher disease: report of a Peruvian case

La enfermedad de Gaucher, es un trastorno autosómico recesivo de depósito lisosomal que se caracteriza por deficiencia de la beta-glucocerebrosidasa que lleva a la acumulación de glucosilceramida principalmente en células del sistema fagocítico mononuclear causando afectaciones sistémicas. Se presenta paciente varón de 20 años que cursa con dolor crónico en hipocondrio izquierdo con episodios de sangrados desde hace 3 años y sensación de alza térmica, al examen físico se identificó ictericia y esplenomegalia masiva, sin afectación neurológica. Como apoyo al diagnóstico se mostró osteoporosis severa, pancitopenia y como hallazgo inesperado la presencia de trombosis de vena porta con transformación cavernomatosa complicada con biliopatía portal simulando un tumor de klatskin, los estudios de médula y enzimáticos eran compatibles con enfermedad de Gaucher, por lo cual recibió tratamiento con imiglucerasa realizando seguimiento. Es un caso poco frecuente, de gran interés, heterogeneidad en sus manifestaciones clínicas e inéditas por su complicación, constituyendo un desafío llegar a su diagnóstico de esta enfermedad huérfana.

Gaucher disease is an autosomal recessive lysosomal storage disorder characterized by deficiency of beta-glucosidase that would lead to the accumulation of glucosylceramide mainly in cells of the mononuclear phagocytic system causing systemic effectations. We present a patient of twenty years who is suffering from chronic pain in the left hypochondrium with episodes of bleeding for 3 years and sensation of thermal rise, physical examination revealed jaundice and massive splenomegaly, without neurological involvement. Severe osteoporosis, pancytopenia, and the presence of portal vein thrombosis with cavernomatous transformation complicated by portal biliopathy simulating a klatskin tumor, marrow and enzymatic studies were compatible with Gaucher disease, were shown as unexpected findings. he received treatment with imiglucerase, following up. It is a rare case, of great interest, heterogeneity in its clinical manifestations and unpublished by its complication, constituting a challenge to reach its diagnosis of this orphan disease.

Colangiopatía portal: complicación poco frecuente de la hipertensión portal / Portal cholangiopathy: infrequent portal hypertension complication / Colangiopatia do portal: complicação incomum da hipertensão portal

Se describe el caso clínico de una mujer de 62 años en la que se diagnostica una estenosis del colédoco en el contexto de hipertensión portal prehepática (cavernomatosis portal).

We report the case of a 62 years old woman with a choledochal stricture and chronic obstruction of the portal vein (portal cavernomatosis).

Relatamos o caso de uma mulher de 62 anos com diagnóstico de estenose do colédoco no contexto da hipertensão portal pré-hepática (cavernomatose portal).

Resultados del tratamiento quirúrgico del colangiocarcinoma intrahepático / Intrahepatic cholangiocarcinoma: experience in 30 operated patients

background: Intrahepatic cholangiocarcinoma (ICC) corresponds to 10% of liver primary malignant tumors. Aim: To show the results of surgical treatment of ICC in a biliary surgery center. material and methods: Review of a prospective database of operated patients at a surgical service of a clinical hospital. Thirty operated patients with an ICC, aged 25 to 83 years (20 women), were identified. Results: Twenty six patients had symptoms, 12 of 19 had high levels of CA19-9 and in four the tumor was non resectable. Twenty four patients underwent major hepatectomy and two, a minor hepatectomy. Combined resections were performed in three patients. Lymphadenectomy was performed in 14 patients and five had lymph node metastases. Surgical time was 272 minutes, mean intensive care unit stay was 10 days and mean ventilatory support use was five days. Surgical mortality was 19% and complications appeared in 53% of patients. Tumors were stage I, II, III and IV in 11, 5, 3 and 11 patients respectively. Overall survival was 16 months. Survival in tumors stage I and II was 50% at five years. In stages III and IV, it was 11.2 months. Conclusions: Surgery for ICC has an acceptable mortality and complications rate with a five years survival of 25%.

Introducción: El colangiocarcinoma intrahepático (CCIH), corresponde al segundo cáncer primario hepático, representando alrededor del 10% de los cánceres primarios del hígado; el tratamiento es la hepatectomía. Objetivo: Mostrar los resultados del tratamiento quirúrgico de pacientes con CCIH en Chile en un centro de cirugía hepatobiliar. Pacientes y métodos: Análisis prospectivo de los pacientes con CCIH operados en nuestro centro entre 2005 y 2015. Resultados: 30 pacientes, 20 mujeres (67%), 10 hombres (33%). Edad promedio: 60 años (rango 25-83 ), 26 pacientes sintomáticos (87%), CA19-9 elevado en 12 (63,2%), 4 pacientes (13%) irresecables, 24 pacientes (80%) hepatectomías mayores y 2 resecciones menores. Resecciones combinadas en 3, linfadenectomía en 14 pacientes (47%), metástasis linfonodales en 5 (17%). Tiempo promedio quirúrgico 272 min (rango 45-480). UCI, mediana 10 días, ventilación mecánica mediana 5 días. Hospitalización, mediana 10 días y 7 días postoperatorio. Morbilidad 53%, mortalidad 19%. R0 en 19 pacientes (64%), R1 en 7 pacientes (23%) y R2 en 4 pacientes (13%). Estadio I en 11 pacientes, estadio II en 5 pacientes, estadio III en 3 pacientes, estadio IV en 11 pacientes. Sobrevida general de 16 meses. Sobrevida estadio I 58,4 meses, estadio II 31,1 meses, estadio III 5,9 meses y estadio IV 11,8 meses, p = 0,06. Sobrevida R0 16 meses, R1: 31,1 meses y R2: 9,25 meses, p = 0,53. Sobrevida estadios I y II 50% a 5 años, estadios III-IV 11,2 meses, p < 0,01. Discusión: Este es el primer reporte nacional de una serie importante de pacientes con CCIH, con morbilidad y mortalidad aceptables. Sobrevida de 25% a 5 años.

Volume-reserving Surgery after Photodynamic Therapy for Biliary Papillomatosis: A Case Report / 대한소화기학회지

Biliary papillomatosis is rare, and its pathogenic mechanisms are not yet clear. Because of its high risk for malignancy transformation, surgical resection is regarded as a standard treatment. Photodynamic therapy (PDT) has been used by the intravenous administration of hematoporphyrin derivative followed by laser exposure. A photochemical process causes disturbance of the microvascular structure and degradation of membrane. Cholangitis is a major complication after PDT. A healthy 56-year-old man was diagnosed with biliary papillomatosis involving the common hepatic duct, both proximal intrahepatic bile ducts (IHD), and the right posterior IHD. After biliary decompression by endoscopic nasobiliary drainage, PDT was performed to avoid extensive liver resection and recurrence using endoscopic retrograde cholangiographic guidance. After portal vein embolization, the patient underwent extended right hemihepatectomy. Following administration of chemoradiation therapy with tegafur-uracil and 45 Gy due to local recurrence at postoperative 13 months, there was no local recurrence or distant metastases. This is the first case report on PDT for biliary papillomatosis in Korea. Preoperative PDT is beneficial for reducing the lesion in diffuse or multifocal biliary papillomatosis and may lead to curative and volume reserving surgery. Thus, PDT could improve the quality of life and prolong life expectation for biliary papillomatosis patients.

Inflammatory Hepatic Adenoma / 대한소화기학회지

No abstract available.

Cancer Stem Cells in Primary Liver Cancers: Pathological Concepts and Imaging Findings

There is accumulating evidence that cancer stem cells (CSCs) play an integral role in the initiation of hepatocarcinogenesis and the maintaining of tumor growth. Liver CSCs derived from hepatic stem/progenitor cells have the potential to differentiate into either hepatocytes or cholangiocytes. Primary liver cancers originating from CSCs constitute a heterogeneous histopathologic spectrum, including hepatocellular carcinoma, combined hepatocellular-cholangiocarcinoma, and intrahepatic cholangiocarcinoma with various radiologic manifestations. In this article, we reviewed the recent concepts of CSCs in the development of primary liver cancers, focusing on their pathological and radiological findings. Awareness of the pathological concepts and imaging findings of primary liver cancers with features of CSCs is critical for accurate diagnosis, prediction of outcome, and appropriate treatment options for patients.

As mucinas e o NCAM (CD56) na colangiocarcinogênese intra-hepática / Mucins and NCAM (CD56) in intrahepatic cholangiocarcinogenesis

Introduction: Cholangiocarcinoma is the second most common malignant neoplasm of the hepatobiliary system. During cholangiocarcinogenesis phenotypic changes occur in the ductal epithelium, including the expression of mucins (MUC). However, the evaluating studies of the expression of mucins in the different stages of cholangiocarcinogenesis are scarce. CD56 has also contributed in differentiating benign ductal proliferation and cholangiocarcinoma; however, its expression has not been evaluated in dysplastic epithelium of the bile duct yet. Objective: To assess immunohistochemical profile of (MUC) 1, 2, 5, 6, and CD56 in cholangiocarcinoma, pre-neoplastic and reactive lesions in the epithelium of intrahepatic bile ducts. Material and methods: Immunohistochemical expression of MUC 1, 2, 5, 6, and CD56 were studied for 11 cases of cholangiocarcinoma and 83 intrahepatic bile ducts (67 reactive and 16 dysplastic). Variables were considered significant when p < 0.05. Results: The expression of MUC1 occurred in about 90% of the cholangiocarcinomas, contrasting with the low frequency of positive cases in reactive and dysplastic bile ducts (p < 0.001). However, there was no statistically significant difference in the expression of MUC5, MUC6 and CD56 between the reactive or dysplastic lesions and cholangiocarcinoma. The anti-MUC2 antibody was negative in all cases. Conclusions: MUC1 contributed for the differential diagnosis between cholangiocarcinoma and pre-neoplastic and reactive/regenerative lesions of intrahepatic bile ducts, and it should compose the antibodies panel aiming at improvement of these differential diagnoses. In contrast, MUC2, MUC5, MUC6 and CD56 were not promising in differentiating all the phases of cholangiocarcinogenesis...

Introdução: O colangiocarcinoma é a segunda neoplasia maligna mais comum do sistema hepatobiliar. Durante a colangiocarcinogênese podem ocorrer alterações fenotípicas do epitélio ductal, incluindo a expressão de mucinas. Entretanto, os estudos que avaliam a expressão das mucinas nas diferentes etapas da colangiocarcinogênese são escassos. O CD56, apesar de contribuir na diferenciação entre as proliferações ductais benignas e o colangiocarcinoma, ainda não teve a sua expressão avaliada no epitélio displásico dos ductos biliares. Objetivos: Analisar o perfil das mucinas (MUC) 1, 2, 5, 6 e do CD56 no colangiocarcinoma, nas lesões pré-neoplásicas e reacionais de ductos biliares intra-hepáticos. Material e métodos: A expressão imuno-histoquímica da MUC 1, 2, 5, 6 e do CD56 foram avaliadas em 11 colangiocarcinomas e 83 ductos biliares intra-hepáticos (67 reativos e 16 displásicos). As variáveis foram consideradas como significativas quando p < 0,05. Resultados: A expressão da MUC1 ocorreu em cerca de 90% dos colangiocarcinomas, contrastando com a baixa frequência de casos positivos nos ductos biliares reativos ou displásicos (p < 0,001). Não houve diferença estatisticamente significativa na expressão de MUC5, MUC6 e CD56 entre as lesões reativas, displásicas e o colangiocarcinoma. O anticorpo anti-MUC2 foi negativo em todos os casos. Conclusão: A MUC1 contribuiu no diagnóstico diferencial entre o colangiocarcinoma e as lesões pré-neoplásicas e reacionais/regenerativas dos ductos biliares intra-hepáticos, e deve compor o painel de anticorpos a ser empregado visando o aprimorando destes diagnósticos diferenciais. Contrariamente, a MUC2, MUC5, MUC6 e o CD56 não se mostraram promissoras na diferen...

Synchronous Malignant Intraductal Papillary Mucinous Neoplasms of the Bile Duct and Pancreas Requiring Left Hepatectomy and Total Pancreatectomy / 대한소화기학회지

Intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) and intraductal papillary mucinous neoplasm of the pancreas (IPMN-P) have striking similarities and are recognized as counterparts. However, simultaneous occurrence of IPMN-B and IPMN-P is extremely rare. A 66 year-old female presented with recurrent epigastric pain and fever. During the past 9 years, she had three clinical episodes related to intrahepatic duct stones and IPMN-P in the pancreas head and was managed by medical treatment. Laboratory test results at admission revealed leukocytosis (12,600/mm3) and elevated CA 19-9 level (1,200 U/mL). Imaging study demonstrated liver abscess in the Couinaud's segment 4, IPMN-B in the left lobe, and IPMN-P in the whole pancreas with suspicious malignant change. Liver abscess was drained preoperatively, followed by left lobectomy with bile duct resection and total pancreatectomy with splenectomy. On histologic examination, non-invasive intraductal papillary mucinous carcinoma arising from various degree of dysplastic mucosa of the liver and pancreas could be observed. However, there was no continuity between the hepatic and pancreatic lesions. This finding in our case supports the theory that double primary lesions are more likely explained by a diffuse IPMN leading to synchronous tumors arising from both biliary and pancreatic ducts rather than by a metastatic process. Herein we present a case of simultaneous IPMN of the bile duct and pancreas which was successfully treated by surgical management.

Intrahepatic cholangiocarcinoma arising in Caroli's disease

No abstract available.

Acute Obstructive Cholangitis Complicated by Tumor Migration after Transarterial Chemoembolization: A Case Report and Literature Review / 대한소화기학회지

Intraductal tumor invasion of hepatocellular carcinoma (HCC) is considered rare. Transarterial chemoembolization (TACE) is effective for tumor thrombus of HCC in the bile duct. However, a few cases of obstructive jaundice caused by migration of a tumor fragment after TACE have recently been reported. The aim of this study was to identify factors that affect tumor migration after TACE. At this writing, a review of the medical literature disclosed seven reported cases of biliary obstruction caused by migration of a necrotic tumor cast after TACE. We, herein, report on an additional case of acute obstructive cholangitis complicated by migration of a necrotic tumor cast after TACE for intrabile duct invasion of HCC, in a 71-year-old man. The tumor cast in the common bile duct was removed successfully using a basket during ERCP and was pathologically confirmed to be a completely necrotic fragment of HCC. The patient's symptoms showed dramatic improvement. In summary, physicians should be aware of acute obstructive cholangitis complicated by tumor migration in a patient undergoing TACE. We suggest that an intrabile duct invasion would be a major predisposing factor of tumor migration after TACE and drainage procedures such as ERCP or percutaneous transbiliary drainage could be effective treatment modalities in these patients.

Triple-Tissue Sampling during Endoscopic Retrograde Cholangiopancreatography Increases the Overall Diagnostic Sensitivity for Cholangiocarcinoma

BACKGROUND/AIMS: There are several methods for obtaining tissue samples to diagnose malignant biliary strictures during endoscopic retrograde cholangiopancreatography (ERCP). However, each method has only limited sensitivity. This study aimed to evaluate the diagnostic accuracy of a combined triple-tissue sampling (TTS) method (on-site bile aspiration cytology, brush cytology, and forceps biopsy). METHODS: We retrospectively reviewed 168 patients with suspicious malignant biliary strictures who underwent double-tissue sampling (DTS; n=121) or TTS (n=47) via ERCP at our institution from 2004 to 2011. RESULTS: Among the 168 patients reviewed, 117 patients (69.6%) were eventually diagnosed with malignancies. The diagnostic sensitivity for cancer was significantly higher in the TTS group than the DTS group (85.0% vs 64.9%, respectively; p=0.022). Furthermore, the combination of brush cytology and forceps biopsy was superior to the other method combinations in the DTS group. With respect to cancer type (cholangiocarcinoma vs noncholangiocarcinoma), interestingly, the diagnostic sensitivity was higher for cholangiocarcinoma in the TTS group than the DTS group (100% vs 69.4%, respectively; p<0.001) but not for the non-cholangiocarcinoma patients (57.1% vs 57.1%, respectively). CONCLUSIONS: TTS can provide an improved diagnostic accuracy in suspicious malignant biliary strictures, particularly for cholangiocarcinoma.

A Case of IgG4 Associated Sclerosing Cholangitis without Clinical Manifestations of Autoimmune Pancreatitis / 대한소화기학회지

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.

Prognostic significance of snail expression in hilar cholangiocarcinoma

Many patients with hilar cholangiocarcinoma (HC) have a poor prognosis. Snail, a transcription factor and E-cadherin repressor, is a novel prognostic factor in many cancers. The aim of this study was to evaluate the relationship between snail and E-cadherin protein expression and the prognostic significance of snail expression in HC. We examined the protein expression of snail and E-cadherin in HC tissues from 47 patients (22 males and 25 females, mean age 61.2 years) using immunohistochemistry and RT-PCR. Proliferation rate was also evaluated in the same cases by the MIB1 index. High, low and negative snail protein expression was recorded in 18 (38%), 17 (36%), and 12 (26%) cases, respectively, and 40.4% (19/47) cases showed reduced E-cadherin protein expression in HC samples. No significant correlation was found between snail and E-cadherin protein expression levels (P = 0.056). No significant correlation was found between snail protein expression levels and gender, age, tumor grade, vascular or perineural invasion, nodal metastasis and invasion, or proliferative index. Cancer samples with positive snail protein expression were associated with poor survival compared with the negative expresser groups. Kaplan-Meier curves comparing different snail protein expression levels to survival showed highly significant separation (P < 0.0001, log-rank test). With multivariate analysis, only snail protein expression among all parameters was found to influence survival (P = 0.0003). We suggest that snail expression levels can predict poor survival regardless of pathological features and tumor proliferation. Immunohistochemical detection of snail protein expression levels in routine sections may provide the first biological prognostic marker.