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.

Resección hepática extendida con vena cava inferior por colangiocarcinoma intrahepático: presentación de 2 casos / Extended hepatic resection with inferior cava vein for intrahepatic cholangiocarcinoma: presentation of two cases

Resumen Introducción: El colangiocarcinoma intrahepático (CIH) corresponde al segundo tumor hepático primario y la resección quirúrgica es la única alternativa válida para el tratamiento curativo de esta enfermedad. Reporte de casos: Describimos 2 paciente portadores de CIH con compromiso de vena cava inferior (VCI) que fueron sometidos a resección en Clínica Alemana de Santiago (CAS). Ambas pacientes son de género femenino de 39 y 47 años de edad. Ambas fueron sometidas a resección mayor hepática izquierda, asociada a resección del segmento I y extendida a VCI. La reconstrucción de la VCI fue realizada con parche pericárdico bovino y cierre primario respectivamente. El período desde el posoperatorio hasta el alta, fue de 13 y 23 días respectivamente. Discusión: Aunque la reseccion quirúrgica es la única vía para la curación en el CIH, el compromiso de estructuras vasculares hacen que esto no sea posible. El manejo multidisciplinario asociado a una técnica meticulosa realizada por un equipo quirúrgico experimentado, hacen posible lograr buenos resultados.

Introduction: Intrahepatic cholangiocarcinoma is the second most common primary liver tumor and surgical resection the only valid curative treatment. Case reports: We describe two patients harboring an intrahepatic cholangiocarcinoma with cava vein involvement who underwent resection at Clinica Alemana of Santiago. Both patients were females with ages of 39 and 47 years old. Both patients underwent left liver resection, associated to resection of segment I and of a portion of cava vein. Reconstruction of resected portion of the cava vein was performed by using a pericardium bovine patch and primary closure respectively. Postoperative period was uneventfully being discharged at 13 and 23 days respectively. Discussion: Although surgical resection is the only way to get curativeness, frequent involvement of large vascular structures make treatment unfeasible. A multidisciplinary approach associated with a meticulous technique performed by an experienced surgical team make possible to accomplish the above objective.

Ureteropelvic junction obstruction caused by metastatic cholangiocarcinoma

ABSTRACT We describe the rare case of a 61-year-old female with right ureteropelvic junction (UPJ) obstruction caused by metastatic cholangiocarcinoma. Her past medical history was notable for cholangiocarcinoma treated with neoadjuvant chemoradiation and two orthotopic liver transplants six years earlier. Urology was consulted when she presented with flank pain and urinary tract infection. Diagnostic workup demonstrated right UPJ obstruction. She was managed acutely with percutaneous nephrostomy. She subsequently underwent robotic pyeloplasty and intrinsic obstruction of the UPJ was discovered. Histological examination revealed adenocarcinoma, consistent with systemic recurrence of the patient's known cholangiocarcinoma.

Cholangiocarcinoma with a paraneoplastic leukemoid reaction mimicking a pyogenic liver abscess

No abstract available.

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 Adenocarcinoma in situ of the Distal Common Bile Duct Diagnosed by Percutaneous Transhepatic Cholangioscopy

Extrahepatic cholangiocarcinoma is often clinically challenging to diagnose. Even multidisciplinary approaches which include computed tomography, magnetic resonance imaging, and endoscopic retrograde cholangiography are unsatisfactory in some cases, especially with biliary stricture. Percutaneous transhepatic cholangioscopy (PTCS) with its direct visualization for biopsy appears to be a promising technique for detecting cholangiocarcinoma at an early stage. We report a case of adenocarcinoma in situ of the distal common bile duct (CBD) that was confirmed by PTCS. This case suggests the useful role of PTCS in the differential diagnosis of a distal CBD obstruction, particularly when other diagnostic modalities do not provide definitive information.

A case of emphysematous hepatitis with spontaneous pneumoperitoneum in a patient with hilar cholangiocarcinoma / 대한간학회지

An 80-year-old woman with hilar cholangiocarcinoma was hospitalized due to sudden-onset abdominal pain. Computed tomography revealed hepatic necrosis accompanied with emphysematous change in the superior segment of the right liver (S7/S8), implying spontaneous rupture, based on the presence of perihepatic free air. Although urgent percutaneous drainage was performed, neither pus nor fluids were drained. These findings suggest emphysematous hepatitis with a hepatic mass. Despite the application of intensive care, the patient's condition deteriorated rapidly, and she died 3 days after admission to hospital. Liver gas has been reported in some clinical diseases (e.g., liver abscess) to be caused by gas-forming organisms; however, emphysematous hepatitis simulating emphysematous pyelonephritis is very rare. The case reported here was of fatal emphysematous hepatitis in a patient with hilar cholangiocarcinoma.

Percutaneous Biliary Drainage Using Open Cell Stents for Malignant Biliary Hilar Obstruction

OBJECTIVE: To evaluate the feasibility, safety and the effectiveness of the complex assembly of open cell nitinol stents for biliary hilar malignancy. MATERIALS AND METHODS: During the 10 month period between January and October 2007, 26 consecutive patients with malignant biliary hilar obstruction underwent percutaneous insertion of open cell design nitinol stents. Four types of stent placement methods were used according to the patients' ductal anatomy of the hilum. We evaluated the technical feasibility of stent placement, complications, patient survival, and the duration of stent patency. RESULTS: Bilobar biliary stent placement was conducted in 26 patients with malignant biliary obstruction-T (n = 9), Y (n = 7), crisscross (n = 6) and multiple intersecting types (n = 4). Primary technical success was obtained in 24 of 26 (93%) patients. The crushing of the 1st stent during insertion of the 2nd stent occurred in two cases. Major complications occurred in 2 of 26 patients (7.7%). One case of active bleeding from hepatic segmental artery and one case of sepsis after procedure occurred. Clinical success was achieved in 21 of 24 (87.5%) patients, who were followed for a mean of 141.5 days (range 25-354 days). The mean primary stent patency period was 191.8 days and the mean patient survival period was 299 days. CONCLUSION: Applying an open cell stent in the biliary system is feasible, and can be effective, especially in multiple intersecting stent insertions in the hepatic hilum.

Poroceratose superficial disseminada num doente com colangiocarcinoma: manifestação paraneoplásica? / Disseminated superficial porokeratosis in a patient with cholangiocarcinoma: a paraneoplastic manifestation?

As poroceratoses compreendem um grupo de doenças da queratinização epidérmica, hereditárias ou adquiridas, caracterizadas histologicamente pela presença de lamela cornoide. A variante clínica designada por poroceratose superficial disseminada tem sido descrita na literatura, associada a estados de imunossupressão, como transplantação de órgãos, terapêuticas imunossupressoras e infecções. A sua associação a neoplasias sólidas foi raramente descrita na literatura, estando publicados apenas 5 casos. Descrevemos o caso clínico de um paciente que desenvolveu, subitamente, lesões de poroceratose superficial disseminada, concomitantemente ao diagnóstico de um colangiocarcinoma.

Porokeratosis refers to a group of hereditary or acquired disorders of epidermal keratinization and is characterized histologically by the presence of a cornoid lamella. The clinical variant referred to as disseminated superficial porokeratosis has been described in the literature in association with immunosuppressive conditions that include organ transplant, infections and immunosuppressive treatments. The association of disseminated superficial porokeratosis with solid organ malignancies has seldom been described, only 5 such cases having been published. The present report refers to a patient with lesions of disseminated superficial porokeratosis of sudden onset shortly before diagnosis of a cholangiocarcinoma.

Quistes de colédoco en adultos / Choledochal cysts in adults

Introducción: Los quistes de colédoco en adulto son muy raros ya que la mayoría se diagnostica antes de los 10 años de edad. Los síntomas más frecuentes son dolor abdominal e ictericia, principalmente en mujeres. El objetivo de esta investigación fue informar la experiencia en el manejo de quistes de colédoco en adultos en un hospital de tercer nivel. Material y métodos: Estudio retrospectivo, descriptivo, observacional y transversal de la evolución posoperatoria de pacientes adultos con diagnóstico de quistes de colédoco tratados en un servicio de cirugía general en un periodo de 17 años. Resultados: Fueron en total 23 pacientes, de los cuales 16 fueron mujeres, con una mediana de edad de 26 años. Los síntomas principales fueron dolor abdominal (87 %) e ictericia (57 %). Solo cuatro pacientes (17 %) tuvieron además de estos síntomas tumor abdominal. El método diagnóstico más utilizado fue ultrasonido (100 %) seguido de colangiografía endoscópica (78 %) y tomografía computarizada de abdomen (74 %). De acuerdo con la clasificación de Todani, el tipo de quiste fue I en 17 pacientes, II en dos pacientes, III en tres pacientes y IV-A en un paciente. Todos fueron intervenidos quirúrgicamente, a 18 se les realizó resección del quiste y hepatoyeyuno anastomosis, a dos resección del quiste y a tres esfinteroplastia transduodenal. Hubo complicaciones posoperatorias en siete pacientes (35 %) y no hubo mortalidad operatoria. Solo en uno se informó colangiocarcinoma. Conclusiones: La cirugía de elección para el manejo de los quistes de colédoco es la resección del mismo, con morbilidad y mortalidad aceptables.

BACKGROUND: Choledochal cysts are usually diagnosed during childhood. They mainly affect females. Abdominal pain and jaundice are the two most common symptoms. Our objective was to report the experience of a third-level referral center in the management of choledochal cysts in adults. METHODS: We performed a 17-year retrospective review of patients with choledochal cysts who underwent surgical interventions in a gastrointestinal surgery department. RESULTS: There were 23 patients with a median age of 26 years; 16 were females. The most frequent symptoms were abdominal pain and jaundice. Only four patients had an abdominal mass. The most frequently used diagnostic studies were abdominal sonography and endoscopic retrograde cholangiopancreatography. According to the classification of Todani, most cysts were type I and were found in 17 patients, two patients had type II, three patients had type III (choledocele) and one patient had type IVA. Eighteen patients underwent complete removal of the cyst with a concomitant hepatic-jejunostomy, three patients had transduodenal sphincteroplasty and two patients had only cystectomy. Seven patients developed postoperative complications, representing a morbidity rate of 35%. None of the patients died during the first 30 postoperative days. Cholangiocarcinoma was found in the resected specimen in only one case. CONCLUSIONS: The surgery of choice for choledochal cysts in the adult is hepatojejunostomy, which has a low surgical morbidity and mortality.

Hepatectomía derecha extendida por colangiocarcinoma intrahepático en un paciente con síndrome de Dubin-Johnson / Hepatectomy for cholangiocarcinoma in one patient with Dubin Johnson syndrome

Dubin-Johnson syndrome (SDJ) is a rare benign autosomal recessive congenital disease that causes jaundice. We report a 71 years old male with a Dubin Jonson syndrome diagnosed at the age of 40. On a ultrasound examination and magnetic resonance imaging, a liver tumor was found, that was excised. The pathology report showed a cholangiocarcinoma. During the postoperative period, the patient had a hyperbilirubinemia that subsided slowly. There are reports of hyperbilirubinemia of difficult management after hepatectomy in patients with Dubin-Johnson syndrome.

El síndrome de Dubin-Johnson (SDJ) es una patología benigna, familiar, con carácter autosómico recesivo y de aparición poco frecuente. Consiste en un defecto en la excreción hepática de la bilirrubina conjugada que permite su entrada al canalículo biliar, manifestando un cuadro clínico de ictericia silente. La asociación de esta enfermedad con colangiocarcinoma intrahepático es excepcional, siendo difícil la sospecha precoz de este diagnóstico y también el manejo de la ictericia postoperatoria. El objetivo de este trabajo es describir un caso clínico con estas características y revisión de la literatura existente. Se presenta a un paciente de sexo masculino de 71 años de edad, con antecedente de síndrome de Dubin-Johnson diagnosticado a los 40 años. Asintomático, en exámenes de rutina se encuentra un tumor hepático en la ecotomografía abdominal, que luego se corrobora en la TAC y RNM, mide aproximadamente 10 cm de diámetro, de ubicación central y derecho. Resto de estudio no muestra diseminación. Se realiza laparoscopia diagnóstica y ecografía intraoperatoria confirmando lo visto por imágenes, y se decide convertir a laparotomía para efectuar hepatectomía derecha extendida. Biopsia rápida y diferida informan colangiocarcinoma. Evoluciona favorablemente en el postoperatorio, con hiperbilirrubinemia sostenida de lenta resolución. La asociación de hepatectomía con síndrome de Dubin-Johnson es de muy baja frecuencia, existiendo poca literatura al respecto. Se ha descrito que la hiperbilirrubinemia en el postoperatorio es de difícil manejo, sin embargo, en este caso el paciente evolucionó favorablemente sin necesidad de alguna terapia específica.

A Case of Choledochoduodenal Fistula as a Delayed Complication after Biliary Metallic Stent Placement in Distal Cholangiocarcinoma / 대한소화기학회지

Biliary drainage in patients with malignant biliary obstruction relieves jaundice and prevents the development of cholangitis or hepatic failure from biliary obstruction. Therefore, this may result in better quality of life along with survival prolongation. Biliary stent placement is an effective and safe measure for biliary decompression and is preferred than bypass surgery in high risk patients. Entero-biliary perforation-communication is one of the rare complications of biliary stent. We herein report a case of duodeno-biliary perforation-communication in patient with distal cholangiocarcinoma who presented with duodenal ulcer and obstruction, occurring 4 years later from the metallic biliary stent insertion. Patient was managed with a pyloric metal stent and conservative care.

Patología obstructiva de la vía biliar: análisis bacteriológico / Patología obstructiva de la vía biliar: análisis bacteriológico

Introducción: La infección del árbol biliar constituye una de las complicaciones más serias que afectan a pacientes con patología hepatobiliopancreática. El estudio bacteriológico de la bilis, sumado a la fisiopatología de obstrucción canalicular y su realización con diferentes parámetros, permite instrumentar mediante la confección de protocolos de procedimientos, distintas conductas terapéuticas. Objetivo: Analizar nuestra experiencia en el estudio bacteriológico de la bilis en los enfermos con patología obstructiva canalicular. Lugar de aplicación: Práctica hospitalaria y extrahospitalaria. Diseño: Observacional retrospectivo. Material y métodos: Entre julio de 2002 y febrero de 2003, se analizaron 97 pacientes con Síndrome de Hipertensión canalicular de diversa etiología. En todos los casos se efectúo drenaje percutáneo de la vía biliar como tratamiento de la obstrucción canalicular y se obtuvo, como primer gesto, bilis para cultivo y antibiograma. Resultados: El análisis de las muestras enviadas a cultivo demostró desarrollo bacteriano en el 42,2% de los pacientes (41 cultivos positivos). Los cultivos fueron positivos en el 37,5% de los pacientes con patología maligna (30 casos). Se obtuvo desarrollo bacteriano en el 65% de los pacientes con enfermedad benigna (11 casos), siendo la Klebsiella el micro organismo más frecuente, seguido de enterococo y E. coli. El 41% de los pacientes con Tumor de Klatskin tuvieron cultivos positivos. Por otra parte, en el 86% de los pacientes con instrumentación biliar percutánea previa, el cultivo fue positivo para enterococo y E. coli. Conclusión: Consideramos que ante un paciente con patología obstructiva de la vía biliar de cualquier etiología en quien se efectúa una instrumentación percutánea debe realizarse profilaxis antibiótica y antibioticoterapiagía en quien se fectúa una instrumentación percutánea debe realizarse profilaxis antibiótica y antibioterapia inicial en los grupos de riesgo...

Intrahepatic Cholangiocarcinoma associated with Hepatolithiasis / 대한간학회지

No abstract available.

Trousseau's Syndrome in Association with Cholangiocarcinoma: Positive Tests for Coagulation Factors and Anticardiolipin Antibody

Thromboembolic events are reported to occur with a high frequency in the setting of malignancy. However, reports on an association between cholangiocarcinoma and pulmonary thromboembolism, thus far, are almost lacking. We present here an unusual case of a 56-yr-old patient presenting cholangiocarcinoma and unexplained pulmonary thromboembolism. The patient had been quite healthy before the diagnosis. Coagulation tests showed elevated levels of fibrinogen, fibrinogen degradation product (FDP), D-dimer, and IgM anticardiolipin antibody (aCL Ab). The thromboemboli were resolved 3 weeks after anticoagulant therapy using lowmolecular-weight-heparin. Then, follow-up coagulation tests showed a marked decrease to normal in aCL Ab titer as well as the normalization of FDP and D-dimer levels. In this case, we describe pulmonary thromboembolism caused by hypercoagulable state associated with cholangiocarcinoma and speculate that such a thrombotic phenomenon could be regressed by anticoagulant therapy.

A Case of Hepatic Inflammatory Pseudotumor Developed with Peripheral Cholangiocarcinoma / 대한소화기학회지

Inflammatory pseudotumor is an uncommon mass which develops most frequently in the lung of young adults. It is characterized by localized fibrous proliferations with chronic inflammatory cell infiltration. Due to its rarity and similarity in radiologic appearance with malignant hepatic tumors, hepatic inflammatory pseudotumor (HIPT) is often misdiagnosed and resected accidentally. We report a case of HIPT which was unnecessarily resected due to synchronous small peripheral cholangiocarcinoma located on the other segment of liver.

Postoperatorio crítico de un paciente con colangiocarcinoma y cirrosis / Critical postoperative of a patient with cirrhosis and cholangiocarcinoma

We present a patient that has alcoholic hepatic failure and cholangiocarcinoma, morbity obesity and sickness apnea that has a post surgery complication with severe sepsis,acute renal failure, hepatic failure and septic shock for nosocomial pneumonia. The principal complication was acute renal failure that requires continious hemodiafiltration, for 3 weeks approximately, and intermittent hemodialisis for 5 weeks more. The patient survive with no chronical complication and has a normal performance.

Mucinous Cholangiocarcinoma associated with Clonorchis sinensis Infestation: A Case Report / 대한간학회지

Mucinous cholangiocarcinoma, characterized by large quantities of mucin production, is a rare subtype of peripheral cholangiocarcinoma and usually shows rapid progression and a fatal outcome. We report here a case of mucinous cholangiocarcinoma in a 69 year-old man, who was infected with Clonorchis sinensis. Histologically, the tumor was an adenocarcinoma with extensive intracellular and extracellular mucin production, up to 70% of the tumor mass and there was frequent lymphovascular invasion of the tumor cells. The liver adjacent to the mass contained eggs of Clonorchis sinensis in the bile duct lumen and showed ductal epithelial hyperplasia, mucinous metaplasia and adenomatous proliferation of intramural glands. The patient was treated with a right hepatectomy. Four months after the surgery, the tumor recurred in the soft tissue of the right flank.