Cholangiocarcinoma incidence and survivalIncidence of intrahepatic cholangiocarcinoma is increasing in the United States1
aAnnual percentage change (APC).
- Cholangiocarcinoma is the second most common primary liver cancer and is associated with a poor prognosis1,2
- Overall incidence: 1.2 to 2.2 per 100,0001,3,4
- 53.6% to 66.9% are intrahepatic cholangiocarcinomas1,3
- Most patients (63%) are >65 years at diagnosis1
- Largely due to a lack of specific symptoms, most patients (~70%) are diagnosed at late stages when the disease is unresectable2
Prognosis For Cholangiocarcinoma
The 5-year relative survival rate for metastatic CCA is 2%5
IDH1 mutations are common genetic alterations in cholangiocarcinomamIDH1 is an early driver mutation that occurs in up to 20% of intrahepatic CCA cases in the United States6,7
Biomarker testing for mIDH1 can be performed by next generation sequencing of tumor tissue using an FDA-approved companion diagnostic8
Biomarker testing combined with targeted therapy could improve survival outcomes in cholangiocarcinoma8
TIBSOVO® (ivosidenib tablets) selectively inhibits mIDH1 and may play an important role in filling an unmet need in the treatment of advanced cholangiocarcinoma8,9
IDH1, isocitrate dehydrogenase-1; mIDH1, mutated IDH1.
IDH1 mutations and cholangiocarcinomaIDH1 mutations can play a critical role in the development of cholangiocarcinoma
IDH1 mutations confer gain-of-function activity10
- Isocitrate dehydrogenase (IDH) is a key enzyme in the citric acid cycle that catalyzes the conversion of isocitrate to α-ketoglutarate (α-KG)11
- Cancer-associated mutations in IDH1 catalyze the reduction of α-KG to the oncometabolite 2-hydroxyglutarate (2‑HG)10
- Accumulation of 2-HG leads to oncogenesis
- Elevated levels of 2-HG have been observed across multiple tumors with IDH1/2 mutations, including acute myeloid leukemia, glioma, and cholangiocarcinoma10,12,13
NCCN Guidelines® Recommendations for CCARecommendations from the NCCN Clinical Practice Guidelines in Oncology15
- “Given emerging evidence regarding actionable targets for treating cholangiocarcinoma, molecular testing of unresectable and metastatic tumors is recommended.”b
- Ivosidenib (TIBSOVO) is recommended as a subsequent-line treatment option for unresectable or metastatic cholangiocarcinoma with an IDH1 mutation following disease progressionb
bTo view the most recent and complete version of the guidelines, visit NCCN.org.