Abstract
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 surgery and 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 and treatment of cholangiocarcinoma.
Highlights
Холангиокарцинома (ХГК) представляет собой группу злокачественных опухолей с признаками дифференцировки в направлении эпителия желчных путей
Мутации определялись в онкогенных и супрессорных генах – TP53, KRAS (16.7%), и семействе SMAD4 (16.7%)
Barr Fritcher EG, Kipp BR, Voss JS, et al Primary sclerosing cholangitis patients with serial polysomy fluorescence in situ hybridization results are at increased risk of cholangiocarcinoma.Am J Gastroenterol.2011; Vol.106:2023–2028
Summary
ФУНДАМЕНТАЛЬНАЯ ОНКОЛОГИЯ И ЭКСПЕРИМЕНТАЛЬНАЯ МЕДИЦИНА FUNDAMENTAL ONCOLOGY AND EXPERIMENTAL MEDICINE. ВХГК – наиболее частый тип ХГК, в больших сериях пациентов с ХГК – лишь 8% приходится на ВПХГК, а на ВХГК и ДХГК – 50% и 42%, соответственно [4]. На ХГК приходится порядка 3% всех опухолей ЖКТ, но за последние 20–30 лет этот процент неуклонно растет [6], при этом пятилетняя выживаемость сохраняется на уровне 10% [7, 8]. 10–20% летальных исходов от опухолей печени и желчных путей приходится на ХГК [9]. В соответствии с базой данных SEER (США), частота встречаемости для ВПХГК выросла с 0.59/100000 в 1990 г. Частота встречаемости ВХГК и ДХГК («опухолями Клацкина»), напротив, оставалась в пределах 0.8/100000 до 2001 г., и подросла до 0.97 в 2007 г. Введение новой классификации – ICD-O-3 объясняет изменения частоты встречаемости ХГК в разных странах – например, в Великобритании в 2008 г. [6, 16]
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