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Hilar cholangiocarcinoma, also called perihilar cholangiocarcinoma or Klatskin tumor, is defined in the packet as a cholangiocarcinoma that arises from the junction, or adjacent to the junction, of the right and left hepatic ducts. The packet lists Orphanet and GARD (NIH) among its sources. Prevalence is categorized as uncommon, with an estimate of 1-9 in 100,000; the packet does not state the measure type or population for this estimate. The affected organ system listed is the digestive system.
The packet lists no associated genes, OMIM entries, or inheritance patterns. The causes and genetic basis are therefore unknown in this packet, and no gene or inheritance claims are made here.
The packet contains no diagnostic methods, diagnostic criteria, or GeneReviews diagnosis content, so diagnostic approaches are not described here.
The packet includes one orphan-drug record with status APPROVED: Lytgobi (futibatinib), sponsor Taiho Oncology, Inc., with the indication recorded as treatment of cholangiocarcinoma (CCA). The record does not specify current market status, tumor subtype restrictions, or eligibility, so these details are unknown in this packet. The packet certifies no foundational therapies or GeneReviews management content, so other treatment approaches are not described here.
23 trials found
Prognosis is not certified in this packet. It contains no natural history or survival information, and none is stated here.
ClinicalTrials.gov records in the packet include several studies that name perihilar or hilar cholangiocarcinoma. NCT04993131, titled "Liver Transplantation for Non-resectable Perihilar Cholangiocarcinoma" (Oslo University Hospital), is listed as recruiting, with a start date of 2021-09-01 and a completion date of 2045-05-31. NCT07030140, a phase 2 study titled "Phase II Study of Neoadjuvant Tislelizumab Plus Radiotherapy and GP Chemotherapy for Borderline/Unresectable Hilar Cholangiocarcinoma" (sponsor Jinbo Yue), is listed as recruiting, 2025-07-01 to 2028-01-01. NCT05546372, "Endobiliary Radiofrequency Ablation for Malignant Biliary Obstruction Due to Perihilar Cholangiocarcinoma" (Academisch Medisch Centrum - Universiteit van Amsterdam), is listed as recruiting, 2022-10-22 to 2027-06-30. Some other records are listed as not yet recruiting, and their current status is not verified here. Trial titles do not establish intervention effects.
Data assembled from 5 of 12 sources · Last updated Nov 3, 2026, 10:00 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
AI-curated news mentioning hilar cholangiocarcinoma
Updated Oct 12, 2026
A case report details a right anterior sectionectomy performed for a hepatic helminthic infection that was initially misdiagnosed as cholangiocarcinoma. This highlights the importance of accurate diagnosis in rare liver infections.
A case study highlights the cholangioblastic variant of intrahepatic cholangiocarcinoma in a young adult with situs inversus totalis. This discovery adds to the understanding of rare cancer presentations and their potential associations.
Research from the GLOBAL-BTC registry reveals that chronic liver disease correlates with earlier-stage diagnosis of cholangiocarcinoma and better prognosis. These findings could influence future screening and treatment strategies for patients.
FDA approves Bizengri (zenocutuzumab-zbco) for treating NRG1 fusion-positive cholangiocarcinoma, marking the seventh approval under the National Priority Voucher Pilot Program. This ultra-rare cancer affects the bile ducts and has limited treatment options.