Onco Wiki / Disease

Cholangiocarcinoma (bile duct cancer)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-CHOLANGIOCARCINOMA
TypeDisease
Aliases
Bile duct carcinomaCholangiocarcinomaKlatskin tumor (perihilar)dCCAiCCApCCAХолангіокарцинома (рак жовчних проток)
Statusreviewed 2026-04-27 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-NCCN-HCC-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology8160/3
icd_o_3_topography["C22.1", "C24.0", "C24.8"]
icd_10C22.1, C24.0
who_classificationWHO Digestive System Tumours 5th ed (2019): Intrahepatic / Perihilar / Distal cholangiocarcinoma

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Notes

Three anatomically and biologically distinct subtypes; actionable alterations cluster in intrahepatic. Resectable: surgery + adjuvant capecitabine 6 months (BILCAP). Liver transplant for selected pCCA after Mayo neoadjuvant protocol (chemo-radiation + brachy → exam-under-anesthesia → transplant). Advanced 1L: gemcitabine + cisplatin + durvalumab (TOPAZ-1) replaced gem-cis monotherapy as standard. Pembrolizumab + gem-cis is an alternative (KEYNOTE-966). Advanced 2L+ by genotype (intrahepatic CCA enrichment): - FGFR2 fusion / rearrangement (~10–20% iCCA): pemigatinib (FIGHT-202) or futibatinib (FOENIX-CCA2) — both FDA-approved. Hyperphosphatemia is on-target effect. - IDH1 R132 (~15% iCCA): ivosidenib (ClarIDHy) — modest PFS benefit. - HER2 IHC 3+ / amplified (~5–10% perihilar/distal): trastuzumab + pertuzumab (MyPathway) or trastuzumab deruxtecan (DESTINY-PanTumor02); zanidatamab (HERIZON-BTC-01). - BRAF V600E (~5%): dabrafenib + trametinib (ROAR — tumor-agnostic). - MSI-H / dMMR (~2–3%): pembrolizumab (KEYNOTE-158). - NTRK fusion (rare): larotrectinib / entrectinib. Bile-duct decompression / drainage management is parallel concern; cholangitis / biliary stent dysfunction is the mo...

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