Learn About Malignant Peripheral Nerve Sheath Tumor

What is the definition of Malignant Peripheral Nerve Sheath Tumor?
A Malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops in the protective lining that covers nerves. The first symptom of MPNST is often a lump or mass that increases in size, sometimes causing pain or a tingling sensation. MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung. Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.
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What are the alternative names for Malignant Peripheral Nerve Sheath Tumor?
  • Malignant peripheral nerve sheath tumor
  • Malignant neurilemmoma
  • Malignant neurofibroma
  • Malignant schwannoma
  • Neurofibrosarcoma
  • Neurogenic sarcoma
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What are the latest Malignant Peripheral Nerve Sheath Tumor Clinical Trials?
A Phase 3 Randomized Study of Selumetinib Versus Carboplatin/Vincristine in Newly Diagnosed or Previously Untreated Neurofibromatosis Type 1 (NF1) Associated Low-Grade Glioma (LGG)

Summary: This phase III trial studies if selumetinib works just as well as the standard treatment with carboplatin/vincristine (CV) for subjects with NF1-associated low grade glioma (LGG), and to see if selumetinib is better than CV in improving vision in subjects with LGG of the optic pathway (vision nerves). Selumetinib is a drug that works by blocking some enzymes that low-grade glioma tumor cells need ...

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A Phase II Study of Binimetinib in Children and Adults With NF1 Associated Plexiform Neurofibromas (PNOC010)

Summary: This is a phase II open label study that will evaluate children ≥ 1 year of age and adults with neurofibromatosis type 1 (NF1) and plexiform neurofibromas treated with the MEK inhibitor, binimetinib. The primary objective is to determine if there is an adequate level of disease responsiveness to binimetinib in children and adults with NF1 and inoperable plexiform neurofibromas. The objective respo...

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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Malignant Peripheral Nerve Sheath Tumor?
A case report on a novel use of intraoperative Intrabeam™ radiation therapy for a recurrent malignant peripheral nerve sheath tumor with sciatic nerve involvement.
Visual Outcomes After Treatment for Sporadic Optic Pathway Gliomas in Pediatric Patients: A Systematic Review.
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Successful Radiotherapy of Primary Malignant Peripheral Nerve Sheath Tumor of the Lung.