2226-5988 2686-6749 Клиническая и экспериментальная морфология Clinical and Experimental Morphology ООО "Группа МДВ" 10.31088/CEM2026.15.1.13-22 Внутрипеченочная холангиокарцинома Intrahepatic cholangiocarcinoma Багрецова Мария Андреевна Багрецова Мария Андреевна Bagretsova Maria A. bagretsova.pathology@gmail.com 0009-0005-2884-9713 Паклина Оксана Владимировна Паклина Оксана Владимировна Paklina Oxana V. 0000-0001-6373-1888 Карнаухов Николай Сергеевич Карнаухов Николай Сергеевич Karnaukhov Nikolay S. 0000-0003-0889-2720 Абрамов Иван Сергеевич Абрамов Иван Сергеевич Abramov Ivan S. 0000-0002-6954-1564 ГБУЗ Московский клинический научно-практический центр имени А.С. Логинова Департамента здравоохранения города Москвы, Москва, Россия The Loginov Moscow Clinical Scientific Center, Moscow, Russia ГБУЗ Московский клинический научно-практический центр имени А.С. Логинова Департамента здравоохранения города Москвы, Москва, Россия; ФГБУ Национальный медицинский исследовательский центр хирургии имени А.В. Вишневского Минздрава России, Москва, Россия The Loginov Moscow Clinical Scientific Center, Moscow, Russia; A.V. Vishnevsky National Medical Research Center of Surgery, Moscow, Russia ГБУЗ Московский клинический научно-практический центр имени А.С. Логинова Департамента здравоохранения города Москвы, Москва, Россия; Центр «Генетическое репрограммирование и генная терапия» ГБУЗ Московского клинического научно-практического центра имени А.С. Логинова Департамента здравоохранения города Москвы, Москва, Россия The Loginov Moscow Clinical Scientific Center, Moscow, Russia; Genetic Reprogramming and Gene Therapy Center, The Loginov Moscow Clinical Scientific Center, Moscow, Russia 27 02 2026 15 1 13 22 30 10 2025 10 12 2025 25 11 2025

Внутрипеченочная холангиокарцинома является второй по распространенности первичной карциномой печени после гапатоцелюллярного рака. В последнее время отмечается тенденция к увеличению частоты данной патологии. На основе клинико-морфологических особенностей, мутационного профиля и прогноза внутрипеченочные холангиокарциномы делят на два подтипа: новообразования из мелких протоков и новообразования из крупных протоков. Течение заболевания и прогноз у двух подтипов резко отличаются друг от друга. Для мелкопротоковой внутрипеченочной холангиокарциномы свойственны мутации IDH1/2 и FGFR2-fusion, в то время как для внутрипеченочной холангиокарциномы из крупных протоков более характерны мутации KRAS и TP53. Целью обзора является анализ современных данных о гистопатологической классификации внутрипеченочной холангиокарциномы, её молекулярных особенностях, характеристиках опухолевого микроокружения и достижениях в области геномных исследований.

Intrahepatic cholangiocarcinoma is the most common primary liver cancer after hepatocellular carcinoma. The incidence of intrahepatic cholangiocarcinoma has been increasing lately. Based on clinical and morphological features, mutational profile, and prognosis, intrahepatic cholangiocarcinomas are divided into two subtypes (small duct type and large duct type), whose course and prognosis differ significantly. The former is characterized by IDH1/2 mutations and FGFR2 fusions, while the latter is marked by KRAS and TP53 mutations. This review aimed to analyze current data on the histopathological classification of intrahepatic cholangiocarcinoma and its molecular features, characteristics of tumor microenvironment, and study advances in genomic research.

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