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El colangiocarcinoma hiliar, comúnmente conocido como tumor de Klatskin, es una neoplasia poco frecuente. Su etiopatogenia no es completamente conocida, pero se encuentra una fuerte asociación entre la inflamación crónica de la vía biliar, la colestasis y el desarrollo de colangiocarcinoma. Los principales factores de riesgo son la colangitis esclerosante primaria, las enfermedades fibroquísticas del hígado, infecciones hepáticas, hepatolitiasis, drogas y toxinas. Este tumor se caracteriza por presentar un cuadro clínico asintomático hasta estadíos avanzados. Su diagnóstico se realiza por medio de laboratorios e imagenología. El tratamiento se basa principalmente en cirugía, ya que no hay beneficios terapéuticos demostrados con el uso de radiación y/o quimioterapia.
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