Table 2.

Immunotherapy of chordomas with CTLA-4 immune checkpoint inhibitor.

Year	Study Design	Levels of Evidence	Sample Size	Tumor Site	Treatment History	Drug	Median Treatment Time (m)	AEs	Choi’s Criteria	RECIST/irRECIST	Median PFS (m)	Median OS (m)
22	case series	IV	5			Durvalumab + tremelimumab	12	Colitis, pneumonitis, abdominal pain, myocarditis		1PD,3SD,1PR
21	phase1/2	V	1			INT230-6 + ipilimumab		grade1/grade2, anemia, colitis
19	case report	V	1	sacrum	Radiotherapy	Nivolumab, followed by nivolumab + ipilimumab					>4

Abbreviations: NR, not reported; PR, partial response; SD, stable disease; PD, progressive disease.