Neoplasms

All Conditions

Symptoms and General Pathology

Nervous System Diseases

Skin and Connective Tissue Diseases

Diseases and Abnormalities at or Before Birth

Rare Diseases

Sarcoma

Recurrence

Nerve Sheath Neoplasms

Neurofibrosarcoma

Neoplasms, Nerve Tissue

Neoplasms by Histologic Type

Nervous System Neoplasms

Peripheral Nervous System Neoplasms

Peripheral Nervous System Diseases

Neuromuscular Diseases

Fibrosarcoma

Neoplasms, Fibrous Tissue

Neoplasms, Connective Tissue

Neoplasms, Connective and Soft Tissue

Neurofibromatoses

Neurofibroma

Soft Tissue Sarcoma

Malignant Peripheral Nerve Sheath Tumor

Neurofibromatosis

Subjects will receive 520 mg/m2/dose (subjects 12-17 years old) or 800 mg of tazemetostat orally twice daily in 28 day cycles. Subjects will receive treatment with tazemetostat up to 2 years or until disease progression or unacceptable toxicity occurs.

Tazemetostat

Priya Gurjar

Ashley Bayne

Danielle Geckler

Joanne Lagmay, MD

This phase 2, open label, single arm study will investigate the use of tazemetostat in patients with recurrent/refractory and/or metastatic malignant peripheral nerve sheath tumors.

Tazemetostat in Malignant Peripheral Nerve Sheath Tumors

Phase 2 Study Using Tazemetostat in Patients With Recurrent/Refractory and/or Metastatic Malignant Peripheral Nerve Sheath Tumors (MPNST)

Assess the objective response rate, defined as the proportion of subjects who achieve either a complete response or partial response based on radiographic evaluation of treatment response via RECIST1.1

Determine the progression free survival, defined as the length of time from the start of treatment to time of progression or death, whichever occurs first.

Determine the time to progression, defined as the length of time from the start of treatment until disease progression by RECIST 1.1 criteria.

Determine the clinical benefit using the Numbered Pain Rating Scale. A person rates their pain on a scale of 0 to 10 or 0 to 5. Zero means "no pain," and 5 or 10 means "the worst possible pain." These pain intensity levels may be assessed upon initial treatment, or periodically after treatment.

Assess the clinical benefit rate, defined as the proportion of subjects with complete or partial response or stable disease (by RECIST 1.1 criteria) lasting at least 4 months.

Epizyme, Inc.

University of Florida

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
Tazemetostat in Malignant Peripheral Nerve Sheath Tumors	NCT04917042	Peripheral Nerv...	Tazemetostat	12 Years - 99 Years	University of Florida
Tazemetostat in Malignant Peripheral Nerve Sheath Tumors	NCT04917042	Peripheral Nerv...	Tazemetostat	12 Years - 99 Years	University of Florida

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