Table 3.

Clinical trials and reports exploring the use of CPIs in SWI/SNF-deficient sarcomas, clear cell sarcomas and angiosarcomas.

Year/Author	Trial	Agents	Rare Subtypes Evaluable (n)	ORR of Rare Subtype	Other Outcomes
2020 Geoerger [63]	KEYNOTE-051 phase I-II	Pembrolizumab	MRT (2) EPS (1)	50% MRT 100% EPS
2021 Blay [52]	AcSé Pembrolizumab phase II	Pembrolizumab	MRT (11)	27%	18.2% 12-month PFS rate (MRT)
2019 Jones [69]	MD Anderson case series	CPI	CCS (11)	NR	1/11 Durable response (41.8 months) 10/11 <6 months response duration
2020 Painter [70]	Angiosarcoma project series	CPI	AS (6)	33%	2 CR (2/3 HNFS AG)
2020 Somaiah [21]	MD Anderson phase II	Durvalumab and tremelimumab	AS (5)	20%	1 PR (1/1 cutaneous AS)
2019 Florou [71]	Miami Miller case series	Pembrolizumab, AGEN1884, Pembrolizumab and axitinib	AS (7)	NR	71% PR rate at 12 weeks 1/2 CR to AGEN1884
2021 Wagner [72]	DART phase II	Ipilimumab and nivolumab	AS (16)	25%	60% HNFS AS ORR 2 SD 38% 6-month PFS rate

Abbreviations: AS, angiosarcoma; CCS, clear cell sarcoma; CPI, checkpoint inhibitor; CR, complete response; EPS, epithelioid sarcoma; HNFS, head, neck, face, and scalp; MRT, malignant rhabdoid tumour; NR, not recorded; ORR, objective response rate; PFS, progression-free survival; PR, partial response; SD, stable disease.