Neoplasms

Nervous System Diseases

All Conditions

Skin and Connective Tissue Diseases

Diseases and Abnormalities at or Before Birth

Rare Diseases

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

Sarcoma

Neurofibromatoses

Neurofibroma

Malignant Peripheral Nerve Sheath Tumor

Soft Tissue Sarcoma

Neurofibromatosis

Antineoplastic Agents

All Drugs and Chemicals

Decitabine

Decitabine and cedazuridine drug combination

Antimetabolites

Patients who meet the eligibility criteria will be treated with oral ASTX727 (INQOVI) on days 1-5 of each 21-day cycle with Pegfilgrastim support on day 7. A delay in the start of subsequent cycles due to holidays, weather, or other circumstances will be permitted up to 7 days and not considered a protocol deviation. Drug dosing will be interrupted for any Grade 4 adverse events or clinically significant laboratory abnormalities. For Grade 3 or 4 AE, if the AE returns to Grade 1 or baseline, the patient may be re-escalated.

ASTX727 will be self-administered orally by the patient on a once daily basis, days 1 through 5 of each 21-day cycle. Cycle 1 day 1 (C1D1) is defined as the first day that ASTX727 is administered.

ASTX727

Ping Chi, MD, PhD

Ciara Kelly, MBBCH BAO

The purpose of this study is to see whether the study drug ASTX727 is an effective treatment for people who have MPNST with a PCR2 mutation.

ASTX727 is a combination of two drugs (cedazuridine and decitabine) that have been designed to target cancer cells with a PCR2 mutation and to disrupt the cells' ability to survive and grow. The study researchers think that the study drug allows decitabine to work better than decitabine given alone.

This is a phase II trial to evaluate the efficacy of ASTX727 in patients with PRC2-loss MPNST.

A Study of ASTX727 in People With Malignant Peripheral Nerve Sheath Tumors (MPNST)

A Phase II Study of ASTX727 in Patients With PRC2 Loss Malignant Peripheral Nerve Sheath Tumor (MPNST)

Memorial Sloan Kettering Cancer Center supports the international committee of medical journal editors (ICMJE) and the ethical obligation of responsible sharing of data from clinical trials. The protocol summary, a statistical summary, and informed consent form will be made available on clinicaltrials.gov when required as a condition of Federal awards, other agreements supporting the research and/or as otherwise required. Requests for deidentified individual participant data can be made beginning 12 months after publication and for up to 36 months post publication. Deidentified individual participant data reported in the manuscript will be shared under the terms of a Data Use Agreement and may only be used for approved proposals. Requests may be made to: crdatashare@mskcc.org.

(complete response \[CR\] + partial response \[PR\] + stable disease \[SD\]) by RECIST1.1

Astex Pharmaceuticals, Inc.

Memorial Sloan Kettering Cancer Center

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization	Link
A Study of ASTX727 in People With Malignant Peripheral Nerve Sheath Tumors (MPNST)	NCT04872543	Malignant Perip...	ASTX727	18 Years -	Memorial Sloan Kettering Cancer Center
A Study of ASTX727 in People With Malignant Peripheral Nerve Sheath Tumors (MPNST)	NCT04872543	Malignant Perip...	ASTX727	18 Years -	Memorial Sloan Kettering Cancer Center

Spots Global Cancer Trial Database for prc2 mutation