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Home > Epithelioid Sarcoma

Epithelioid sarcoma is an extremely rare form of soft tissue sarcoma1,2

Epithelioid sarcoma comprises two different subtypes that differ in histology and disease progression3
  • Distal-type (classical): more favorable outcomes observed in patients4
  • Proximal-type: more aggressive course with less favorable outcomes4

Epithelioid sarcoma predominantly affects younger adults in their 20‑30s, although it may occur at any age and is more frequent in men.4

Patients with metastatic epithelioid sarcoma have a poor prognosis5,6

Median overall survival for epithelioid sarcoma patients with metastatic disease is approximately 8 to 12 months.5,6

References: 1. Jawad MU, Extein J, Min ES, and Scully SP. Prognostic factors for survival in patients with epithelioid sarcoma: 441 cases from the SEER database. Clin Ortho Relat Res. 2009;467(11):2939‑2948. 2. van Geel AN, Pastorino U, Jauch KW, et al. Surgical treatment of lung metastases: The European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group study of 255 patients. Cancer. 1996;77(4):675-682. 3. Siontis BL, Chugh R, and Schuetze SM. The potential of emerging therapeutics for epithelioid sarcoma. Expert Opin Orphan Drugs. 2017;5(12):983-989. 4. Sobanko JF, Meijer L, Nigra TP. Epithelioid Sarcoma: A Review and Update. J Clin Aesthet Dermatol. 2009;2(5):49–54. 5. Frezza AM, Jones RL, Lo Vullo S, et al. Anthracycline, gemcitabine, and pazopanib in epithelioid sarcoma: A multi-institutional case series. JAMA Oncol. 2018;4(9):e180219. 6. Spillane AJ, Thomas JM, and Fisher C. Epithelioid sarcoma: The clinicopathological complexities of this rare soft tissue sarcoma. Ann Surg Oncol. 2000;7(3):218‑225.

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Learn more about how TAZVERIK works.

Mechanism of action


TAZVERIK is indicated for the treatment of adults and pediatric patients aged 16 years and older with metastatic or locally advanced epithelioid sarcoma not eligible for complete resection.

This indication is approved under accelerated approval based on overall response rate and duration of response. Continued approval for this indication may be contingent upon verification and description of clinical benefit in a confirmatory trial(s).


Warnings and Precautions

  • Secondary Malignancies

The risk of developing secondary malignancies is increased following treatment with TAZVERIK. Across clinical trials of 729 adults who received TAZVERIK 800 mg twice daily, myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML) occurred in 0.7% of patients. One pediatric patient developed T‑cell lymphoblastic lymphoma (T‑LBL). Monitor patients long-term for the development of secondary malignancies.

  • Embryo-Fetal Toxicity

Based on findings from animal studies and its mechanism of action, TAZVERIK can cause fetal harm when administered to pregnant women. There are no available data on TAZVERIK use in pregnant women to inform the drug‑associated risk. Administration of tazemetostat to pregnant rats and rabbits during organogenesis resulted in dose‑dependent increases in skeletal developmental abnormalities in both species beginning at maternal exposures approximately 1.5 times the adult human exposure (area under the plasma concentration time curve [AUC0‑45h]) at the 800 mg twice daily dose.

Advise pregnant women of the potential risk to a fetus. Advise females of reproductive potential to use effective contraception during treatment with TAZVERIK and for 6 months after the final dose. Advise males with female partners of reproductive potential to use effective contraception during treatment with TAZVERIK and for 3 months after the final dose.

Adverse Reactions

In 62 clinical study patients with epithelioid sarcoma receiving TAZVERIK 800 mg twice daily: Serious adverse reactions occurred in 37% of patients who received TAZVERIK. Serious adverse reactions occurring in ≥3% were hemorrhage, pleural effusion, skin infection, dyspnea, pain, and respiratory distress. The most common (≥20%) adverse reactions were pain (52%), fatigue (47%), nausea (36%), decreased appetite (26%), vomiting (24%), and constipation (21%).

Drug Interactions

Avoid coadministration of strong or moderate CYP3A inhibitors with TAZVERIK. If coadministration of moderate CYP3A inhibitors cannot be avoided, reduce TAZVERIK dose.

Avoid coadministration of moderate and strong CYP3A inducers with TAZVERIK, which may decrease the efficacy of TAZVERIK.

Coadministration of TAZVERIK with CYP3A substrates, including hormonal contraceptives, can result in decreased concentrations and reduced efficacy of CYP3A substrates.


Because of the potential risk for serious adverse reactions from TAZVERIK in the breastfed child, advise women not to breastfeed during treatment with TAZVERIK and for one week after the final dose.

Before prescribing TAZVERIK, please read the full Prescribing Information.