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Sarcoma

Success targeting EZH2 in epithelioid sarcoma may lead to push for personalized medicine in sarcoma management

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Success targeting EZH2 in epithelioid sarcoma may lead to push for personalized medicine in sarcoma management

Targeting EZH2 in epithelioid sarcoma showed significant activity and may establish the benefit of targeting oncogenic drivers within each histological subtype of soft tissue sarcomas, according to a study published in Precision Medicine.

Diagnosing epithelioid sarcoma is challenging, with a key feature being a lack of SMARCB1/INI1 nuclear expression on immunohistochemistry staining.

“Molecular profiling is becoming a necessity to discern the different histological subtypes and to guide the therapeutic sequencing,” the authors wrote. “Due to their rare incidence and the absence of dedicated clinical trials, specific recommendations and guidelines on the optimal management of ES are almost inexistent.”

The researchers hope this data will encourage researchers to look for potential targetable oncogenic drivers within each histological subtype.

In the NTC02601950 multicentric, open-label phase 2 trial, tazemetostat (800 mg) was evaluated in 62 adult patients with locally advanced or metastatic epithelioid sarcoma harboring INI1 loss.

The overall response rate and disease control rate were 15% and 26%, respectively. Median overall survival and median progression free survival were 82.4 weeks and 23.7 weeks. The median duration of response was not reached at a median of 59.9 weeks follow.

Reference
Assi T, Rassy E, Nassereddine H, Farhat F, Kattan J. Epithelioid sarcoma as the prototype for personalized therapy in soft tissue sarcoma. Per Med. 2020;17(4):241-244. doi:10.2217/pme-2020-0006.

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