RESUMO
We present the case of forty-four-year-old woman with autosomal dominant Polycystic liver disease (PLD) without kidney disease diagnosed for 18 years ago. She experienced progressive abdominal distension and malnutrition over the previous twelve months. The patient had no other comorbidities. Physical examination revealed an 8 cm hepatomegaly in the right hypochondrium region. Liver function analysis was normal. CT scan showed multiple liver cysts with smooth and regular walls, predominantly in the right lobe which was considered as type III according to Gigot´s classification. Due to the significant hepatomegaly, conventional surgery was performed. Exploration showed multiple cysts of varying sizes on the liver surface, with diffuse involvement of the right hemiliver. Standard right lobe mobilization was technically not possible. Fenestration was performed in dominant superficial cysts, to allow access to the deeper cysts and liver parenchyma. A right hepatectomy was carried up using ultrasound devices and ultrasonic aspirator. And the biggest cysts located in the surface of left hepatic lobe were surgically unroofed. No surgical complications occurred, and the patient was discharged 5 days after the intervention.
RESUMO
Primary neuroendocrine tumors (NETs) of the bile duct are extremely rare and represent only 0.2-2% of all gastrointestinal NETs. Within the biliary system, the main bile duct is the most affected site. A 28-year-old man with a 6-month history of intermittent jaundice, pruritus, and choluria. MRCP, PET-CT and endoscopic ultrasound were performed. A well-differentiated neuroendocrine neoplasia was diagnosed. Complete resection of the main bile duct was performed with lymphadenectomy of the hepatic pedicle with Roux-en-Y hepaticojejunostomy, without complications. The patient had an adequate evolution and nowadays he's disease-free. Primary neuroendocrine tumors of the bile duct are extremely rare. They may present clinically and radiologically similar to perihilar cholangiocarcinoma, which makes preoperative diagnosis difficult. Radical resection is indicated. Usually, they are well differentiated tumors, being the Ki-67 labeling index a reliable prognostic marker.
Los tumores neuroendocrinos (TNE) primarios de la vía biliar son extremadamente raros y representan sólo el 0.2-2% de todos los TNE gastrointestinales. Dentro del sistema biliar, la vía biliar principal es el sitio más afectado. Hombre de 28 años con cuadro de 6 meses de evolución caracterizado por ictericia intermitente, prurito y coluria. Se realizó colangiopancreatoresonancia magnética nuclear, PET-TC y ultrasonido endoscópico que concluyeron neoplasia neuroendocrina bien diferenciada. Se realizó resección completa de la vía biliar principal con linfadenectomía del pedículo hepático con hepaticoyeyunoanastomosis en Y de Roux, sin complicaciones. El paciente cursó adecuada evolución y se encuentra libre de enfermedad. Los tumores neuroendocrinos primarios de la vía biliar son extremadamente raros, presentándose clínica y radiológicamente como lesiones similares al colangiocarcinoma perihiliar lo que dificulta el diagnóstico preoperatorio. Está indicado su tratamiento quirúrgico radical. Suelen ser bien diferenciados, siendo el antígeno Ki-67 un marcador pronóstico confiable.
Assuntos
Neoplasias dos Ductos Biliares , Neoplasias do Ducto Colédoco , Tumores Neuroendócrinos , Masculino , Humanos , Adulto , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/cirurgia , Neoplasias do Ducto Colédoco/cirurgia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Neoplasias dos Ductos Biliares/cirurgia , FígadoRESUMO
Cholecystocolonic fistula (CCF) is the second most common cholecystoenteric fistula, associated in most cases with stone disease. Symptoms are usually minimal or nonspecific, and preoperative diagnosis is uncommon. Although the incidence of FCC caused by gallbladder cancer comprises 1.7% of cases, it is necessary to suspect it in order to adopt the best therapeutic strategy.
La fístula colecistocolónica (FCC) es la segunda fístula colecistoentérica más común, asociada en la mayoría de los casos a enfermedad litiásica. Los síntomas suelen ser mínimos o inespecíficos, siendo infrecuente su diagnóstico preoperatorio. Si bien la incidencia de FCC causada por cáncer de vesícula biliar comprende el 1,7 % de los casos, es necesario sospecharla para adoptar la mejor estrategia terapéutica.