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Karolak et al. J Cancer Metastasis Treat 2021;7:15 https://dx.doi.org/10.20517/2394-4722.2021.05 Page 13 of 16

Table 2. A summary table of the STS highlighted in this review and the effects of EZH2 modulation for each
STS subtype Drug tested Anti-tumour effect? RNAi phenotype
ERMS DZNep Yes Differentiation
GSK126 Yes; with TPA
MC1945/1948 Yes
ARMS DZNep Yes Proliferation inhibition, initiation of apoptosis
Tazemetostat No
MC1945/1948 Yes
Hybrid EZH2i+HDACi Yes
MRT DZNep Yes; with etoposide, 5-Aza-CdR, SAHA Reduced growth, SMARCB1 recovery
Tazemetostat Yes (if INI1 deficient)
GSK126 Modest; with HDACi
GSK343 Modest
UNC1999 Modest
ATRT DZNep Yes (in-vitro only) Proliferation reduction, initiation of apoptosis
GSK126 Yes; with JQ1
Synovial sarcoma Tazemetostat Yes (if INI1 deficient) Proliferation inhibition
Synergy with doxorubicin
Variable trial results
EPZ011989 Yes
EPZ005687 Yes
Epitheliod sarcoma Tazemetostat Yes (if INI1 negative) No data
MPNST DZNep Yes Growth inhibition, increased apoptosis
Leiomyosarcoma EPZ011989 Yes; with dactolisib No data
Liposarcoma GSK126 Yes; with 5-Aza-dC Proliferation inhibition, initiation of differentiation
GSK343 Yes

effective course of treatment for many STS.

DECLARATIONS
Authors’ contributions
Made substantial contributions to conception and design of this review: Karolak M, Walters ZS
Original draft preparation: Karolak M, Walters ZS
Writing, review, and editing of manuscript: Karolak M, Tracy I, Shipley J, Walters ZS
Performed literature research: Karolak M
Preparation of figures: Tracy I, Walters ZS

Availability of data and materials
Not applicable.

Financial support and sponsorship
This work was supported by a Children with Cancer UK grant (Walters Z S) and by Sarcoma UK (Shipley J,
Walters ZS).