Advances in the management of cholangiocarcinoma

Table 1 Targetable genomic alterations in cholagiocarcinoma under investigation

Alterations	iCCA	pCCA/dCCA	Products under investigation
FGFR fusion	15%-20%	< 5%	Pemigatinib1, Derantinib (ARQ-087), Infigrantinib1 (BGJ398), Erdafitinib, TAS-120, ADZ4547
IDH1/2 mutation	20%	< 5%	Ivosidenib1, Enasidenib (AG-221), BAY 1436032, IDH305
ErbB2 (HER-2) amplification	< 5%	10%-15%	Trastuzumab, iapatinib, TAS0728, A166, PRS-343, ZW25
BRAF mutation	5%	< 5%	Dabrafenib + trametinib
DNA damage repair gene mutation (ARID1A, BRCA1/2)	25%	10%-15%	PARP inhibitors (olaparib, rucaparib)

¹FDA approved.

iCCA: Intrahepatic cholangiocarcinoma; pCCA: Perihilar cholangiocarcinoma; dCCA: Distal cholangiocarcinoma; FGRR: Fibrobast growth factor receptor; IDH: Isocitrate dehydrogenase; ERBB (HER-2): A subtype of epidermal growth factor receptor tyrosine kinase; BRAF: Gene for serine/threonine-protein kinase B-Raf; ARID1A: Gene encoding a swItch/sucrose non-fermentable ATP-dependent chromatin remodeling complex; BRCA: Breast cancer gene.