tumorsMalignant tumoursMalignant tumours2224-50572587-6813Rosoncoweb10.18027/2224-5057-2015-3-3-17tumors-147Research ArticleФУНДАМЕНТАЛЬНАЯ ОНКОЛОГИЯ И ЭКСПЕРИМЕНТАЛЬНАЯ МЕДИЦИНАFUNDAMENTAL ONCOLOGY AND EXPERIMENTAL MEDICINEХолангиоцеллюлярная карцинома сегодня. Литературный аналитический обзорCholangiocellular Cholangiocellular carcinoma today. Literature reviewРОТИНД. Л.ROTIND. L.

д.м.н., заведующий патологоанатомическим отделением

Doctor of medical sciences, head of the Pathomorphologic Department

D.rotin@mknc.ruМосковский Клинический Научный Центр Департамента Здравоохранения г. МосквыРоссияMoscow Clinical Scientific CenterRussian Federation20152610201503317Copyright © РОТИН Д.Л., 20152015РОТИН Д.Л.ROTIN D.L.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.malignanttumors.org/jour/article/view/147

Холангиокарцинома (ХГК) представляет собой группу злокачественных опухолей с признаками дифференцировки в направлении эпителия желчных путей. Анатомически ХГК подразделяют на внутрипеченочные, воротные и дистальные. Данные подтипы различаются не только по локализации, но и по эпидемиологии, этиологии, патогенезу и лечению. Частота встречаемости и смертность от ХГК за последние десятилетия существенно выросли, в то время как выживаемость остается низкой. В разных географических областях вариации ХГК обусловлены различными факторами риска. В последние годы накоплены данные по генетическим изменениям клеток ХГК, кроме того обнаружено что строма этой опухоли содержит множество карциномазависимых фибробластов (КЗФ), стимулирующих развитие и рост опухоли. В характеристике и понимании онкогенезавнутрипеченочной ХГК и воротной ХГК отмечается существенный прогресс. Пациентов с внутрипеченочной ХГК обычно лечат хирургически. Для воротной ХГК основным методом лечения является трансплантация печени с неоадъювантной химиотерапией. Предлагаем обзор современного понимания эпидемиологии, патогенеза, классификации, взглядов на диагностику и лечение ХГК

Cholangiocarcinoma is a group of malignant tumors with signs of differentiation to bile ducts epithelium direction. There are three anatomical types of cholangiocarcinoma: intrahepatic, portal and distal. These types of tumors are different not only anatomically but have different epidemiology, etiology, pathogenesis and treatment.Morbidity and mortality of cholangiocarcinoma have dramatically increased last few decades whereas survival remains low. Variations of cholangiocarcinoma are caused by different factors of risks in different geographic locations. There is information obtained in last few years about genetic transformations of cells of cholangiocarcinoma; moreover, there are carcinoma-dependent fibroblasts revealed in the stroma of cholangiocarcinoma that are stimulating growth of the tumor. There is substantial progress obtained in the understanding of oncogenesis of intrahepatic and portal cholangiocarcinoma. Patients with intrahepatic cholangiocarcinoma usually undergo resectional surgeryand neoadjuvant treatment with liver transplantation serve as a treatment of choice in cases of portal cholangiocarcinoma. This article is a review of the current understanding of the epidemiology, pathogenesis, classification, points of views on the diagnostics andtreatment of cholangiocarcinoma.

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The authors declare that there are no conflicts of interest present.