Perihilar Cholangiocarcinoma (PHCH)

Overview

Perihilar cholangiocarcinoma (pCCA, also called Klatskin tumor) arises from the bile duct epithelium at the hepatic hilum, where the left and right hepatic ducts converge. It is classified in OncoTree under CHOL (Cholangiocarcinoma) and represents the most common anatomic subtype of cholangiocarcinoma (~50–60% of cases). It is distinct from intrahepatic (IHCH) and distal extrahepatic (EHCH) subtypes in its anatomic location, surgical approach (typically requiring hepatectomy with biliary reconstruction), and molecular profile.

Cohorts in the corpus

No primary cohort studies specific to PHCH have been processed yet.

Recurrent alterations

  • FXR (NR1H4) expression is heterogeneous across pCCA/dCCA subtypes (H-score <120), suggesting DNMT-inhibitor combinations may be required to activate FXR-mediated tumor suppression. PMID:25608663
  • ICGC CCA cohort (n=489) includes perihilar tumors among its anatomical breakdown (intrahepatic, perihilar, distal extrahepatic), with survival and alteration data on 459 samples; perihilar CCA spans multiple molecular clusters with anatomical class distinct from but overlapping intrahepatic molecular subtypes. PMID:28667006

Subtypes

  • Perihilar CCA is anatomically classified as a distinct subtype of extrahepatic cholangiocarcinoma, alongside distal eCCA. The bile-acid-active vs bile-acid-inactive molecular subtyping (derived from single-cell RNA-seq) applies across CCA subtypes including pCCA. PMID:25608663

Therapeutic landscape

Sources

This page was processed by entity-page-writer on 2026-05-15.