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.

Fasciolosis intrahepática que simulaba un colangiocarcinoma: Reporte de un caso infrecuente / Intrahepatic fasciolosis that simulated a cholangiocarcinoma: Report an infrequent case

La fasciolosis es una infección parasitaria causada por 2 tipos de fasciola, siendo la más común de tipo hepática, la forma infectante son las metacercarias. Su prevalencia radica en zonas de nivel socioeconómicas bajo y zonas ganaderas. El objetivo es presentar un caso de presentación atípica en el que se evitó someter al paciente a un tratamiento quirúrgico innecesario. Presentamos un caso de un paciente masculino de 69 años que presentó Colangitis, su ecografía y tomografía evidenció dilatación de la vía biliar, por lo que se le realizó una con la colangiopancreatografía retrograda endoscópica (CPRE) evidenciándose una estenosis de colédoco cerca de la bifurcación biliar y una larva de fasciola hepática, la endomicroscopía confocal (EM) no era concluyente, se tomó biopsia de la estenosis que reportó colangiocarcinoma, pero como el paciente no tenía pérdida patológica de peso y marcadores tumorales negativos, se le indicó tratamiento antiparasitario y se realizó una nueva CPRE que evidenció en la EM mucosa inflamatoria de aspecto crónico no tumoral que se confirmó con la nueva biopsia, el paciente tuvo una evolución favorable, de esta forma evitamos un tratamiento no requerido.

Fasciolosis is a parasitic infection caused by 2 types of fasciola, being the most common of hepatic type, the infective form is the metacercariae. Its prevalence lies in low socio-economic zones and livestock areas. The objective is to present a case of atypical presentation in which the patient was avoided to undergo an unnecessary surgical treatment. We present a case of a 69-year-old male patient who presented Cholangitis, his sonography and tomography evidenced dilatation of the bile duct, and one was performed with endoscopic retrograde cholangiopancreatography (ERCP), showing a choledochal stenosis near the bile bifurcation And a liver fasciola larva, confocal endomicroscopy (MS) was inconclusive, biopsy of the stenosis that reported cholangiocarcinoma was reported, but since the patient had no pathological weight loss and negative tumor markers, he was given antiparasitic treatment and was performed A new ERCP that showed in the inflammatory mucosa of chronic non-tumor aspect that was confirmed with the new biopsy, the patient had a favorable evolution, thus avoiding an unnecessary treatment.

Caracterización de los pacientes con tumores de la vía biliar mediante colangiopancreatografía retrógrada endoscópica / Characterization of patients with bile duct tumors by endoscopic retrograde cholangiopancreatography

Introducción: las neoplasias de la vía biliar son infrecuentes y se asocian a alta mortalidad y mal pronóstico. Ocupan el quinto lugar entre los cánceres digestivos después de estómago, colon, recto y esófago. En Cuba existe un incremento en el diagnóstico de esta entidad. Objetivo: caracterizar clínica y endoscópicamente los tumores de la vía biliar de los pacientes. Métodos: se realizó una investigación descriptiva retrospectiva en pacientes que se realizaron colangiopancreatografía retrógrada endoscópica en el Centro de Investigaciones Médico Quirúrgicas entre enero del 2006 y diciembre del 2014. Se revisaron todos los informes de este proceder y se determinaron las variables sociodemográficas (edad y sexo), datos clínicos por lo que se realiza, diagnóstico endoscópico y terapéuticas endoscópicas realizadas. Resultados: se realizaron 830 colangiopancreatografías retrógradas endoscópicas y se diagnosticó tumor de la vía biliar en 73 (8,8 por ciento) pacientes y ampulomas en 40 (4,8 por ciento), predominó el grupo etario entre 60 y 69 años (31,0 por ciento), el 57,8 por ciento del total de pacientes era del sexo femenino, el principal dato clínico que motivó la indicación del proceder fue el síndrome ictérico que estuvo presente en el 76,7 por ciento del total de pacientes y la colocación de endoprótesis fue la terapéutica endoscópica más empleada (80,2 por ciento de los casos). Conclusión: predominaron los pacientes del sexo femenino y la sexta década de la vida, el tumor al nivel del hepático común fue más frecuente, la colangiopancreatografía retrógrada endoscópica desempeñó el papel principal en el diagnóstico y tratamiento paliativo de este grupo de pacientes(AU)

Introduction: neoplasms of the biliary tract are rare and are associated with high mortality and poor prognosis. It ranks fifth among gastrointestinal cancers after stomach, colon, rectum and esophagus. In Cuba there is an increase in the diagnosis of this entity. Objective: to characterize the clinical and endoscopic bile duct tumors of patients. Methods: a retrospective descriptive study in patients was conducted endoscopic retrograde cholangiopancreatography were performed in the CIMEQ Hospital between January 2006 and December 2014. To conduct the study were reviewed all reports of endoscopic retrograde cholangiopancreatography and sociodemographic variables (age and sex), clinical data so that the procedure is performed, endoscopic diagnosis and endoscopic treatment performed type are determined. Results: 830 proceedings of endoscopic retrograde cholangiopancreatography where bile duct tumor was diagnosed in 73 (8.8 percent) patients and ampullary in 40 (4.8 percent) patients, predominant age group between 60 and 69 years to 31,0 percent were performed , the female was present in 57.8 percent of patients, as the main clinical data that motivated the indication of proceeding was the jaundice syndrome was present in 76.7 percent of patients and placement stent was the most used endoscopic therapy in 80.2 percent of cases. Conclusion: patients were predominantly female and the sixth decade of life, the level of the common tumor was more frequent liver, endoscopic retrograde cholangiopancreatography played the leading role in the diagnosis and palliative treatment of this patient group(AU)

A Case of Biliary Cystadenocarcinoma Mistaken for Liver Abscess / 대한소화기학회지

No abstract available.

Caroli's disease misdiagnosed as intraductal papillary neoplasm of the bile duct

Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.

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.

Disappearance of Intrahepatic Bile Duct Hepatocellular Carcinoma after Endoscopic Retrograde Cholangiopancreatography and Transarterial Chemoinfusion: A Case Report / 대한소화기학회지

Invasion of the bile duct by hepatocellular carcinoma (HCC), which is called intrahepatic bile duct HCC, is rare and has a poor prognosis. Early diagnosis and surgical resection is important for treatment. A 58-year-old man who underwent hepatic resection for HCC 4 years ago and received transarterial chemoembolization (TACE) 2 years after the operation for recurred HCC presented with jaundice. CT scan revealed a tumor in the common bile duct without intrahepatic lesion. Therefore, ERCP was done to perform biopsy and biliary drainage. Histological examination was compatible with hepatocellular carcinoma. However, the tumor could not be visualized at angiography and thus, only transarterial chemoinfusion was performed without embolization. The tumor had disappeared on follow-up CT scan, and the patient has been disease free for 23 months without evidence of recurrence. Herein, we report a case of intrahepatic bile duct HCC which disappeared after ERCP.

Clinical Usefulness of Bile Cytology Obtained from Biliary Drainage Tube for Diagnosing Cholangiocarcinoma / 대한소화기학회지

BACKGROUND/AIMS: Biliary drainage is performed in many patients with cholangiocarcinoma (CCA) to relieve obstructive jaundice. For those who have undergone biliary drainage, bile cytology can be easily performed since the access is already achieved. This study aims to determine the clinical usefulness of bile cytology for the diagnosis of CCA and to evaluate factors affecting its diagnostic yield. METHODS: A total of 766 consecutive patients with CCA underwent bile cytology via endoscopic nasobiliary drainage or percutaneous transhepatic biliary drainage from January 2000 to June 2012. Data were collected by retrospectively reviewing the medical records. We evaluated the diagnostic yield of bile cytology with/without other sampling methods including brush cytology and endobiliary forcep biopsy, and the optimal number of repeated bile sampling. Several factors affecting diagnostic yield were then analyzed. RESULTS: The sensitivity of bile cytology, endobiliary forceps biopsy, and a combination of both sampling methods were 24.7% (189/766), 74.4% (259/348), and 77.9% (271/348), respectively. The cumulative positive rate of bile sampling increased from 40.7% (77/189) at first sampling to 93.1% (176/189) at third sampling. On multivariate analysis, factors associated with positive bile cytology were perihilar tumor location, intraductal growing tumor type, tumor extent > or =20 mm, poorly differentiated grade tumor, and three or more samplings. CONCLUSIONS: Although bile cytology itself has a low sensitivity in diagnosing CCA, it has an additive role when combined with endobiliary forceps biopsy. Due to the relative ease and low cost, bile cytology can be considered a reasonable complementary diagnostic tool for diagnosing CCA.

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.

RE: Diffusion-Weighted MRI in Intrahepatic Bile Duct Adenoma Arising from the Cirrhotic Liver

No abstract available.

A Case of Small Cell Neuroendocrine Tumor Occurring at Hilar Bile Duct / 대한소화기학회지

Neuroendocrine tumors of the extrahepatic biliary tree are extremely rare malignancies accounting for 0.2-2.0% of all gastrointestinal carcinoid tumors. Neuroendocrine tumors obstructing the biliary tree are extremely difficult to diagnose preoperatively and nearly impossible to differentiate from cholangiocarcinoma. Statistically, the most common anatomic location in the biliary tree is the common bile duct, followed by the perihilar region. Herein, we present a case of a small cell neuroendocrine carcinoma of the hilum in a 79-year-old man following laparotomy. To our knowledge, this is the first case of small cell type neuroendocrine carcinoma of hilar bile duct reported in Korea.

Primary Tumor Maximum Standardized Uptake Value Measured on 18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography Is a Prognostic Value for Survival in Bile Duct and Gallbladder Cancer / 대한소화기학회지

BACKGROUND/AIMS: Few studies have assessed the prognostic value of the primary tumor maximum standardized uptake value (SUVmax) measured by 2-[18F]-fluoro-2-deoxy-D-glucose PET-CT for patients with bile duct and gallbladder cancer. METHODS: A retrospective analysis of 61 patients with confirmed bile duct and gallbladder cancer who underwent FDG PET-CT in Kangbuk Samsung Medical Center (Seoul, Korea) from April 2008 to April 2011. Prognostic significance of SUVmax and other clinicopathological variables was assessed. RESULTS: Twenty-three patients were diagnosed as common bile duct cancer, 17 as hilar bile duct cancer, 12 as intrahepatic bile duct cancer, and nine as gallbladder cancer. In univariate analysis, diagnosis of intrahepatic cholangiocarcinoma and gallbladder cancer, mass forming type, poorly differentiated cell type, nonsurgical treatment, advanced American Joint Committee on Cancer (AJCC) staging and primary tumor SUVmax were significant predictors of poor overall survival. In multivariate analysis adjusted for age and sex, primary tumor SUVmax (hazard ratio [HR], 4.526; 95% CI, 1.813-11.299), advanced AJCC staging (HR, 4.843; 95% CI, 1.760-13.328), and nonsurgical treatment (HR, 6.029; 95% CI, 1.989-18.271) were independently associated with poor overall survival. CONCLUSIONS: Primary tumor SUVmax measured by FDG PET-CT is an independent and significant prognostic factor for overall survival in bile duct and gallbladder cancer.

Primary Malt Lymphoma of the Common Bile Duct

Primary mucosa-associated lymphoid tissue (MALT) lymphoma arising in the common bile duct (CBD) is extremely rare. In our case of MALT lymphoma, CT and MRI showed long, segmental, irregular wall thickening of the CBD and minimal dilatation of the upstream bile duct. A preoperative diagnosis of cholangiocarcinoma was made, but histologic evaluation confirmed MALT lymphoma of the CBD. We herein present a rare case of MALT lymphoma of the CBD with CT and MRI findings.

Diffusion-Weighted MRI in Intrahepatic Bile Duct Adenoma Arising from the Cirrhotic Liver

A 64-year-old male patient with liver cirrhosis underwent a CT study for hepatocellular carcinoma surveillance, which demonstrated a 1.4-cm hypervascular subcapsular tumor in the liver. On gadoxetic acid-enhanced MRI, the tumor showed brisk arterial enhancement and persistent hyperenhancement in the portal phase, but hypointensity in the hepatobiliary phase. On diffusion-weighted MRI, the tumor showed an apparent diffusion coefficient twofold greater than that of the background liver parenchyma, which suggested that the lesion was benign. The histologic diagnosis was intrahepatic bile duct adenoma with alcoholic liver cirrhosis.

Synchronous Double Primary Hepatic Cancer: Hepatocellular Carcinoma and Intrahepatic Cholangiocarcinoma / 대한소화기학회지

No abstract available.

A Case of Intrahepatic Cholangiocarcinoma Associated with Type IV Choledochal Cyst / 대한소화기학회지

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.

A Case of Colloid Carcinoma Arising in Association with Intraductal Papillary Neoplasm of the Liver / 대한소화기학회지

Colloid carcinoma of the liver is very rare, and its clinicopathologic features have not been well characterized yet. We describe herein a case of colloid carcinoma of the liver. Imaging revealed a lobulated mass, measuring 12 cm in diameter at the right lobe of the liver with direct invasion of adjacent peripheral intrahepatic bile ducts. Right hemihepatectomy of the liver was performed according to the possibility of the tumor's malignant behavior. Histopathological examination of the specimen revealed large extracelluar stromal mucin pools containing floating cuboidal to columnar neoplastic cells without ovarian-like stroma. This case seemed to be colloid carcinoma arising in association with intraductal papillary neoplasm of the liver.

Ictericia obstructiva secundaria a tumor de células granulares de la vía biliar extrahepática / Obstuctive jaundice secondary to extrahepatic bile ducts granular cell tumor

Bile ducts granular cell tumor is a rare entity. Of neural origin, mostly benign, may, however, present mimicking malignancy. We report a 32 years old female presenting with painless jaundice and extrahepatic bile ducts stenosis confirmed with MRC. Extrahepatic bile ducts resection is performed. Reconstruction involves four independent ducts to a Roux en Y enteric loop. She has a good postoperative outcome, with no evidence of complications nor recurrence at 17 months of follow up.

El tumor de células granulares en la vía biliar es una neoplasia rara de origen neural, en su mayoría benigna y cuya presentación puede sugerir patología maligna. Objetivo: Se presenta el caso clínico, características anatomopatológicas, manejo y evolución de una paciente joven que se presenta con ictericia obstructiva por estenosis subcarinal biliar. Paciente y Método: Paciente 32 años, sexo femenino, con ictericia, coluria y prurito. Diagnóstico de estenosis biliar y dilatación de vía biliar intrahepática se confirma con colangiorresonancia magnética. Se realiza resección de vía biliar extrahepática desde supracarinal que incluye vía biliar distal. Reconstitución bilioentérica a Y de Roux que involucra cuatro conductos intrahepáticos. Evoluciona en forma satisfactoria en el postoperatorio. El seguimiento alejado a 17 meses revela una satisfactoria condición de la paciente, sin signos de complicación o recidiva. Conclusión: El manejo por un equipo de experiencia multidisciplinario nos permitió ayudar a una paciente con rara patología, benigna en lo histológico, pero que puede representar un gran desafío técnico.