Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

Teddy Mohamad; Camille Plante; Jean-Philippe Brosseau

doi:10.3390/ijms22168620

Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

Int J Mol Sci. 2021 Aug 10;22(16):8620. doi: 10.3390/ijms22168620.

Authors

Teddy Mohamad¹, Camille Plante¹, Jean-Philippe Brosseau^{1

2}

Affiliations

¹ Department of Biochemistry and Functional Genomics, Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada.
² Centre de Recherche du Centre Hospitalier Universitaire de Sherbrooke, Université de Sherbrooke, Sherbrooke, QC J1E 4K8, Canada.

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) originate from the neural crest lineage and are associated with the neurofibromatosis type I syndrome. MPNST is an unmet clinical need. In this review article, we summarize the knowledge and discuss research perspectives related to (1) the natural history of MPNST development; (2) the mouse models recapitulating the progression from precursor lesions to MPNST; (3) the role of the tumor microenvironment in MPNST development, and (4) the signaling pathways linked to MPNST development.

Keywords: malignant peripheral nerve sheath tumor; mouse models; neurofibroma; neurofibromatosis type I; tumor microenvironment.

Publication types

Review

MeSH terms

Animals
Humans
Nerve Sheath Neoplasms / genetics
Nerve Sheath Neoplasms / metabolism*
Nerve Sheath Neoplasms / pathology
Signal Transduction
Tumor Microenvironment*

Abstract

Publication types

MeSH terms

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