Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft-tissue cancers that arise from peripheral nerves or their protective sheaths and often carry a poor prognosis. They develop when Schwann-lineage cells acquire genetic and epigenetic alterations that disrupt growth control, enabling uncontrolled proliferation, invasion along nerve structures, and metastatic spread; some cases occur in people with neurofibromatosis type 1 or after radiation exposure. In bioengineering, engineered cell systems, three-dimensional tumor models, biomaterials, and microfluidic platforms can help reproduce the tumor microenvironment, examine nerve-tumor interactions, evaluate treatment responses, and support development of more precise therapies.

Malignant Peripheral Nerve Sheath Tumors - Related Videos

Research

JoVE Journal - Medicine
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Orthotopic Xenografting of Human Luciferase-Tagged Malignant Peripheral Nerve Sheath Tumor Cells for in vivo Testing of Candidate Therapeutic Agents

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Cited by 17 •

2011

A method for reliably grafting luciferase-tagged human malignant peripheral nerve sheath tumor cells into the sciatic nerve of immunodeficient mice is described. The use of bioluminescence imaging to demonstrate proper establishment of tumor grafts and criteria for random segregation of animals into study groups are also discussed.

Research

JoVE Journal - Cancer Research

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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2024

We have developed a methodology for assessing whether nervous system neoplasms in genetically engineered mice accurately recapitulate the pathology of their human counterparts. Here, we apply these histologic techniques, defined pathologic criteria, and culture methodologies to neurofibromas and malignant peripheral nerve sheath tumors arising in the P0-GGFβ3 mouse model.

Defining Gene Functions in Tumorigenesis by Ex vivo Ablation of Floxed Alleles in Malignant Peripheral Nerve Sheath Tumor Cells

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2021

Here, we present a protocol for performing gene knockouts that are embryonic lethal in vivo in genetically engineered mouse model-derived tumors and then assessing the effect that the knockout has on tumor growth, proliferation, survival, migration, invasion, and the transcriptome in vitro and in vivo.

Genetic Profiling and Genome-Scale Dropout Screening to Identify Therapeutic Targets in Mouse Models of Malignant Peripheral Nerve Sheath Tumor

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Cited by 1 •

2023

We have developed a cross-species comparative oncogenomics approach utilizing genomic analyses and functional genomic screens to identify and compare therapeutic targets in tumors arising in genetically engineered mouse models and the corresponding human tumor type.

Toluidine Blue Staining of Peripheral Nerve Sections: A Technique to Stain Myelinated Nerve Fibers For Peripheral Nerve Structure Visualization

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2025

This video demonstrates the staining technique of resin-embedded, osmium tetroxide-treated rat peripheral nerve sections using toluidine blue. The stained nerve sections help visualize the fine structures of peripheral nerves.

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