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Tazemetostat for advanced epithelioid sarcoma: current status and future perspectives

Noemi Simeone1, Anna Maria Frezza1, Nadia Zaffaroni2

  • 1Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, 20133, Italy.

Future Oncology (London, England)
|December 8, 2020
PubMed

Insights

Tazemetostat targets EZH2 to treat advanced epithelioid sarcoma (ES). This drug offers a new option for patients with SMARCB1/INI1-deficient ES, though response predictors are still needed.

Area of Science:

  • Oncology
  • Molecular Biology
  • Genetics

Background:

  • Epithelioid sarcoma (ES) is an aggressive, ultra-rare soft-tissue sarcoma characterized by SMARCB1/INI1 deficiency.
  • This deficiency elevates EZH2 expression, a key factor in gene silencing via H3K27me3 methylation.
  • Elevated EZH2 drives cancer cell proliferation in specific sarcoma subtypes.

Purpose of the Study:

  • To evaluate tazemetostat, an EZH2 inhibitor, as a treatment for advanced epithelioid sarcoma.
  • To assess the efficacy of tazemetostat in patients with metastatic or unresectable ES.

Main Methods:

  • A single-arm, Phase II basket study was conducted.
  • Patients aged 16 years and older with advanced or metastatic ES were enrolled.
  • Tazemetostat was administered orally as a treatment.

Main Results:

  • Tazemetostat demonstrated positive results in treating patients with advanced epithelioid sarcoma.
  • The drug is approved for patients with metastatic or advanced ES ineligible for complete resection.
  • Further research is needed to identify clinical and molecular predictors of response.

Conclusions:

  • Tazemetostat represents a novel therapeutic option for epithelioid sarcoma patients.
  • The drug's mechanism involves inhibiting EZH2 and reducing H3K27 methylation.
  • Ongoing investigations explore combinations of tazemetostat with chemotherapy and immunotherapy.
Keywords:
epigeneticsepithelioid sarcomainhibitor of the enhancer of zeste homolog 2integrase interactor 1tazemetostat

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