Diagnostic uncertainty of hepatobiliary cystadenoma: report of 11 cases and review of the literature

Objectives: This research was conducted to highlight the pre-operative diagnostic uncertainty of hepatobiliary cystadenomas and to suggest strategies to improve its diagnostic yield

Methods: All consecutive patients admitted with hepatobiliary cystadenomas from July 2007 to July 2014 were recruited in this study. The following information was retrieved from the medical records of eligible patients: demographics, clinical features, laboratory tests, imaging results, operative procedures, frozen sections, complications, histopathology, outpatient follow-up, and morbidity and mortality

Results: Eleven total patients with a diagnosis of hepatobiliary cystadenomas were treated in our unit. Abdominal ultrasounds and computed tomography [CT] scans were performed in all patients; magnetic resonant imaging [MRI] was performed in three patients with suspicion of hepatobiliary cystadenomas. Six patients underwent a definitive surgery; five patients were incorrectly diagnosed with non-hepatobiliary cystadenoma liver cysts [2 hydatid cysts and 3 simple cysts] by the pre-operative ultrasound and CT scan. These five patients underwent surgical deroofing. The frozen section was positive in two patients and was falsely negative in one patient who was diagnosed with simple cysts. The final histopathology results identified hepatobiliary cystadenomas in all patients

Conclusions: Hepatobiliary cystadenomas are rare and are frequently misdiagnosed as hepatic cystic lesions with resultant inadequate surgical treatments. A diagnosis of hepatobiliary cystadenomas should be considered in all patients with atypical liver cystic lesions. Further pre-operative assessment with MRIs and intra-operative frozen sections may improve the diagnostic yield and provide an opportunity for a definitive radical resection

Rabdomiosarcoma de la vía biliar: Presentación de un caso / Rhabdomyosarcoma of the biliary tree: Case report

Se presenta el caso de una paciente de 21 meses con ictericia recurrente. La consulta inicial se había realizado debido a la aparición de febre de origen desconocido, pero después de 10 días se observó ictericia, acolia y prurito. Se efectuó una colangipancreatografía retrógrada endoscópica con esfnterotomía amplia, con lo que se logró la desaparición de los síntomas. Un mes más tarde, estos reaparecieron, por lo que, con sospecha de que se trataba de un quiste del colédoco, se realizó una nueva colangipancreatografía retrógrada endoscópica para confrmar el diagnóstico y colocar un stent para drenaje de la vía biliar. El material obtenido en el estudio se envió a anatomía patológica y se diagnosticó rabdomiosarcoma embrionario de la vía biliar. Se inició tratamiento con quimioterapia según el protocolo EpSSGRMS 2005. La niña no presentaba metástasis en el momento del diagnóstico. Completó el tratamiento y hasta la fecha de redacción de este trabajo, se encontraba libre de enfermedad.

We present a girl 21 months old with recurrent jaundice. Initially she presented fever of unknown origin but jaundice, white coloured stools and pruritus were observed 10 days later. She underwent endoscopic retrograde cholangiopancreatography with sphincterotomy; symptoms dissapeared. One month later, symptoms came back and, suspecting choledochal cyst the patient underwent endoscopic retrograde cholangiopancreatography for diagnostic confrmation and for placement of a biliary stent. The material obtained was sent for histopathology study and embryonal rhabdomyosarcoma of the biliary tree was diagnosed. The patient started chemotherapy following EpSSGRMS 2005 protocol. There was no evidence of metastasis. She completed treatment and to the day of this report she is free of illness.

Estudo prospectivo e comparativo do escovado obtido pela CPER à ecoendoscopia associada à punção aspirativa com agulha fina (EE-PAAF) no diagnóstico diferencial das estenoses biliares / Prospective comparative study of ERCP brush cytology and EUS-FNA for the diferential diagnosis of biliary strictures

OBJETIVO: Avaliar o desempenho diagnóstico da citologia obtida pela CPER, aquele obtido pela EE-PAAF e a concordância entre patologistas gerais (PG) e especialistas (PE) em pacientes com estenose biliar. MÉTODOS: Incluímos pacientes com estenose biliar identificados pela CPER. A EE-PAAF foi realizada apenas em áreas com efeito de massa ou da parede espessada do ducto biliar. O padrão-ouro foi a cirurgia, histologia e/ou o seguimento. As amostras teciduais foram consideradas: malignas, suspeitas, atípicas, insuficientes ou benignas. Os espécimes obtidos por cada método foi interpretado (cego) por um PG e outro PE. RESULTADO: 46 pacientes foram incluídos (37 malignos e 9 benignos). O diagnóstico final foi de tumor pancreático (26), biliar (11), pancreatite crônica (8) e estenose inflamatória do ducto biliar (1). Sensibilidade e acurácia da CPER foram 43,2 por cento e 52,2 por cento para o PG e 51,4 por cento e 58,7 por cento para o PE. Sensibilidade e acurácia da EE-PAAF foi 52,8 por cento e 58,5 por cento para o PG e 69,4 por cento e 73,2 por cento para o PE. A combinação entre a CPER e EE-PAAF demonstrou maior sensibilidade e acurácia para ambos PG (64,9 por cento e 69,6 por cento) e PE (83,8 por cento e 84,8 por cento), respectivamente. CONCLUSÃO: A citologia obtida pelo escovado da via biliar durante a CPER e as amostras teciduais colhidas pela EE-PAAF tem rendimento semelhante para o diagnóstico das estenoses biliares. No entanto, a combinação dos métodos resulta em uma maior acurácia. Além disso, espera-se que a interpretação das amostras ocorra com maior precisão pelo PE se comparado ao PG.

OBJECTIVE: To evaluate and to compare the diagnostic yield of ERCP brush cytology (ERCP) and EUS-FNA in patients with biliary strictures and evaluates the agreement between general pathologists (GP) and expert GI pathologists (GIP) in the final diagnosis of biliary strictures. METHODS: Patients with biliary strictures documented by ERCP were included. Brush cytology was performed and during EUS, only visible mass lesions or localized bile duct wall thickening were aspirated. The gold standard method for diagnosis was surgical histology and/or follow-up. Tissue sampling results were: malignant, suspicious, atypical, insufficiently or benign. Specimens were interpreted by GP and GIP, blinded for prior tests results. RESULTS: 46 patients were included. Final diagnosis was malignancy in 37 (26 pancreatic - 11 biliary) and benign in 9 (8 chronic pancreatitis - 1 common bile duct inflammatory stricture). Sensitivity and accuracy for ERCP brush cytology were 43.2 percent and 52.2 percent for GP and 51.4 percent and 58.7 percent for GIP. Sensitivity and accuracy for EUS-FNA were 52.8 percent and 58.5 percent, respectively for GP and 69.4 percent e 73.2 percent for GIP. In comparison, the combination of brush cytology and EUS-FNA demonstrated higher sensitivity and accuracy for both GP (64.9 percent and 69.6 percent, respectively) and GIP (83.8 percent and 84.8 percent, respectively) and improved agreement with final diagnosis for both (mostly for GIP). CONCLUSION: Both, ERCP brush cytology and EUS-FNA has a similar yield for the diagnosis of biliary strictures. However, the combination of these methods results in an improved diagnostic accuracy. In addition, GIP might be expected to interpret specimens with greater accuracy than GP.

Biliary Cystic Neoplasm: Biliary Cystadenoma and Biliary Cystadenocarcinoma / 대한소화기학회지

Biliary cystic tumors, such as cystadenoma and cystadenocarcinoma, are rare cystic tumors of liver accounting for fewer than 5% of all intrahepatic cysts of biliary origin. Most biliary cystic tumors arise from intrahepatic bile duct and 10-20% arise from extrahepatic bile duct like common hepatic duct, common bile duct, and gallbladder. The first case report of biliary cystic neoplasm in Korea dated back to 1975 by Bae et al, and over 40 cases of cystadenoma and 35 cases of cystadenocarcinoma were reported since then. These tumors usually present in middle-aged women with a mean age of 50 years. Biliary cystadenomas are lined by single layer of cuboidal or columnar epithelium and are very often multilocular with septal or papillary foldings. Over 80% of cystadenoma have dense mesenchymal stroma composed of dense spindle cells, like ovary. The epithelial lining of cystadenocarcinoma exhibits cellular atypia, mitotic activity, and infiltrative growth, but part of lining epithelium retain the feature of cystadenoma, which support the adenoma-carcinoma sequence. The size of tumors varies from 1.5 to 35 cm. Many patients are asymptomatic, except for the presence of palpable mass. When symptoms are present, they include epigastric or right upper quadrant pain or jaundice by enlarged mass. Biliary cystic tumor should be considered when a single or multilocular cystic lesion with papillary infoldings is detected in the liver by computed tomogram (CT) or ultrasound (US). Cystic wall and internal foldings can be seen enhanced by enhanced CT. US reveals a hypoechoic cystic mass with echogenic septation or papillary infoldings. Cystadenocarcinoma should be suspected when there is elevated mass or nodule in the wall or foldings, or thickened cystic wall on CT or US. But it is extremely difficult to differentiate between cystadenoma and cystadenocarcinoma by imaging alone. Increased tumor markers, carcinoembryonic antigen and carbohydrate antigen 19-9, in serum or cystic fluid have been reported in biliary cystic tumor. But tumor markers cannot distinguish cystadenocarcinoma from cystadenoma or both from other cystic lesions of liver. Malignant cells are not usually recovered in patients with cystadenocarcinoma who underwent cystic fluid cytology before and during surgery. The treatment of choice is radical excision of the mass by means of lobectomy or wide tumor excision. Aspiration, marsupialization, and drainage must be avoided. Inadequate excision of both cystadenoma and cystadenocarcinoma may lead to recurrence. Prognosis after complete excision is excellent.

Significances of Serum Level and Immunohistochemical Stain of CA19-9 in Simple Hepatic Cysts and Intrahepatic Biliary Cystic Neoplasms / 대한소화기학회지

BACKGROUND/AIMS: In spite of various diagnostic modalites, biliary cystic neoplasms (biliary cystadenoma and cystadenocarcinoma) remain to be difficult to diagnose preoperatively. Recently, there are some reports that elevated CA19-9 level in serum and/or cystic fluid could be a useful finding in the differential diagnosis of biliary cystic neoplasm. This study aimed to evaluate the expression of CA19-9 and to elucidate its significances in intrahepatic biliary cystic neoplasms and simple hepatic cysts. METHODS: In 8 patients with biliary cystic neoplasms and 6 simple hepatic cysts, symptoms, radiologic and laboratory findings were reviewed retrospectively. In 5 biliary cystic neoplasms (4 bililary cystadenomas, 1 biliary cystadenocarcinoma) and 5 simple hepatic cysts, immunohistochemical stainings for CA19-9 were performed with formalin-fixed, paraffin-embedded tissues. RESULTS: In 8 biliary cystic neoplasms, two cases were suspected as biliary cystadenoma preoperatively and 6 cases could not be distinguished from simple cysts or cholangiocarcinoma preoperatively. In 6 simple hepatic cysts, 3 cases were diagnosed preoperatively and 3 cases could not be distinguished from biliary cystadenoma or pancreatic pseudocyst preoperatively. Expression of CA19-9 in simple hepatic cysts and biliary cystic neoplasms were 80% in both groups. Expression of CA19-9 is not related to the elevated level of CA19-9 in serum. CONCLUSINOS: Our data suggests that the elevated level of CA19-9 in serum may not be helpful in the preoprative diagnosis of biliary cystic neoplasm.

Biliary tract neoplasms.

From January 1993 to December 1995, complete records of patients with biliary neoplasms were analysed. A total of 124 patients were registered. Majority of patients were in the age range of 40-60 years (median 54 years). There were 38 males and 86 females. Histopathologically, adenocarcinoma was the commonest type (59%). Pain, jaundice and lump were noticed in 119, 54 and 77 patients respectively. Fifty six patients had associated gall stones. Ninety patients had metastatic disease at presentation. Majority of them (110/124) had advanced, inoperable disease and therefore were considered for palliative treatment. Only 14 patients (12%) were considered for curative treatment. Of these 14 patients, all the cases underwent surgery, 10 received radiotherapy and 10 received chemotherapy. Follow up was very poor. The survival of 14 patients, who received curative treatment, ranged from 2 months to 44 months with mean of 16 months.

Carcinoma da junçäo do ducto hepático principal / Carcunoma of the junction of the duct hepatic

Os autores apresentam um caso de colangiocarcinoma da junçäo dos ductos hepáticos principais (tumor de Klatskin), que fora tratado com drenagem biliar externa. Discutem os aspectos do diagnóstico e tratamento.

cytological features of stagnant bile in patients with obstructive biliary disorders

Nineteen bile samples were aspirated during endoscopic cholangiography [ERCP] for patients with obstructive jaundice. The patients were assessed clinically, by laboratory investigations, abdominal ultrasonography, ERCP, operative biopsy and by cytology to determine the cause for the obstruction and to evaluate the diagnostic celluiar features of bile cytology obtained during ERCP. The results of 19 cases consisted of 10 malignant cases, 5 benign and 4 atypical smears. The cytological features differentiating benign from malignant lesions gave quite encouraging results with respect to the reliability of diagnosis of bile samples particularly in association with ERCP, sensitivity 76.9%, specificity 87.5% and diagnostic accuracy 82.6%. Such a method can therefore be considered as a simple reliable, rapid method for preoperative evaluation of obstructive jaundice patients and for the early detection of malignancy in screening programs

Neoplasias malignas de vesícula y vias biliares extra-hepáticas. Revisión clínico- Patológica de 81 casos / Malignancies of gallbladder and extrahepatic bile ducts

Se revisan los aspectos clínicos, quirúrgicos y anátomopatológicos de 81 pacientes con neoplasias de vesícula y vías biliares extrahepáticas dividiéndose en cuatro grupos según la localización. Predominó el sexo femenino, la edad promedio fue de 57.05 años, con mayor incidencia entre la quinta y sexta década de la vida. Podemos concluir que los signos y síntomas no son contributorios, al igual que los éxamenes auxiliares. El tipo histológico más frecuente fue el adenocarcinoma, el pronóstico es pésimo ya que la mayoría de los cánceres del sistema hepatobiliar han crecido mas allá de los límites de una resección curativa al momento en que se hacen clínicamente evidentes. Es de hacer notar que más del 80 por ciento de las neoplasias de vesícula biliar tiene litiasis concomitante

La Cirugía biliar y el papel de la citología en el diagnostico y detección de neoplasias de la vía biliar / Biliary surgery and the cytology in diagnosis and neoplasms detection in the pathways biliar

Se realiza el estudio prospectivo de 100 casos consecutivos de bilis vesicular y coledociana tomadas durante el acto operatorio electivo de vías biliares que ingresaron al quirófano con el diagnóstico de colecistitis crónica calculosa. Se obtuvo 3 por ciento de positividad sin falsos positivos. Los PAP que resultaron negativo pero que después se demostró tumor, se trató de neoplasias que no estaban en contacto con bilis. La alta confiabilidad de este procedimiento, hace surgir su utilización rutinaria junto con la metodología de investigación de la pieza operatoria como lo hace la escuela japonesa. El trabajo en equipo multidisciplinario es fundamental.

Lesiones hepáticas asintomáticas: hallazgos ecográficos / Asymptomatic hepatic lesions: ecographic findings

El advenimiento de la ultrasonografía y su aplicación casi rutinaria en la exploración abdominal ha constituido un valioso avance diagnóstico y un complemento de la semiología y de la radiología. En el campo de la Hepatología, el empleo sistemático de la ecografía ha permitido detectar lesiones focales asintomáticas que antes pasaban en su gran mayoría inadvertidas, que no se pesquisaban radiológicamente o que sólo eran demostrables a través del estudio arteriográfico selectivo. Las lesiones hepáticas son quísticas o sólidas. Entre las primeras destacan los quistes biliares simples y los quistes hidatídicos, además del cistoadenoma biliar mucinoso multilocular que es muy raro pero que tiende a malignizarse. Entre las lesiones sólidas asintomáticas susceptibles de encontrarse ecográficamente, el 90% corresponden al adenoma hepatocelular; la hiperplasia nodular focal y el hemangioma hepático. En general los carcinomas hepatocelulares se encuentran en pacientes con hepatomegalia, con compromiso de la función hepática y del estado general y no en sujetos asintomáticos, lo que es excepcional. Además de las lesiones sólidas, antes mencionadas, debe recordarse la existencia de esteatosis hepáticas focales especialmente en alcohólicos

Anastomoses biliodigestivas intra-hepáticas / Intrahepatic bilio-digestive anastomoses

Os autores abordam o problema da obstruçäo da via biliar alta, tanto de origem benigna quanto maligna. Mostram quais os exames complementares para se chegar ao diagnóstico e as indicaçöes cirúrgicas. Finalmente mostram as principais cirurgias utilizadas para alívio da obstruçäo alta, lembrando que os cirurgiöes devem ficar atentos para a necessidade do uso de uma delas em uma parcela significativa de pacientes com icterícia obstrutiva