Pharmacotherapeutic strategies for epithelioid sarcoma: are we any closer to a non-surgical cure?

Figures & data

Table 1. Summary of the main treatments for ES.

Therapy type	Efficacy	References
Chemotherapy regimens
Anthracycline-based chemotherapy	ORR 22%, PFS 6 months	[28]
	ORR 15%, PFS 7.3 months	[29]
Gemcitabine-based chemotherapy	ORR 27%, PFS 4 months	[28]
	ORR 58%(7/12 Pts), PFS 8 months	[31]
Doxorubicin + Ifosfamide	ORR 12.5% (1/8 Pts), PFS 4 months, OS 11 months	[30]
Trabectedin	ORR 33.3% (1/3 Pts), PFS 4 months, OS 11 months	[30]
Vinorelbine	1 Pt – CR for 4 years, 1 Pt – PR for 27 months	Case reports [33,34]
Targeted Therapies
Tazemetostat	ORR 15%; PFS at 12 months 21%	[32,35,36]
Pazopanib	18 Pts – No OR, PFS 3 months	[28,30,37,38]
Dasatinib	ORR 28%(2/7 pts); PFS 7.9 months OS 21% at 2 years	[39]
Sunitinib	1 Pt – SD for 32 months	Case report [40]
Chemotherapy combination with targeted therapy
Doxorubicin + Tazemetostat	Awaiting results of phase 1b/3 randomized trial	NCT04204941
Immunotherapy
Pembrolizumab	1 Pt – PR for 12 months	[41]
Nivolumab	1 Pt – PR for 4 months 1 Pt – PD	[42]
	1 Pt – PD	[43]
Immunotherapy combinations
Ipilimumab + Nivolumab	1 Pt – CR	[44]
Axitinib + Pembrolizumab	1 Pt – PR for 6 months	[45]
Nivolumab + Sunitinib	7 Pts – No OR	[46]
Dasatinib + Ipilimumab	1 Pt – No OR	[47]
Durvalumab + Tazemetostat	Awaiting results of phase 2 CAIRE trial	NCT04705818

Objective Response Rate (ORR), Objective Response (OR), Progression-Free Survival (PFS), And Overall Survival (OS), Pt/Pts – Patient/Patients, PR – Partial Response, CR – Complete Response, PD – Progressive Disease.

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