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Review
. 2021 Aug 10;22(16):8620.
doi: 10.3390/ijms22168620.

Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

Teddy Mohamad  1 , Camille Plante  1 , Jean-Philippe Brosseau  1   2
Affiliations
Review

Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

Teddy Mohamad et al. Int J Mol Sci. .

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.

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Conflict of interest statement

The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results.

Figures

Figure 1
Figure 1
Neurofibroma to MPNST sequence. Schematic illustrating the genetic events driving the neurofibroma to MPNST sequence and the relative expression of markers associated with this transition.
Figure 2
Figure 2
MPNST signaling pathways. Schematic illustrating the main pathways involved in the development/maintenance of MPNSTs (mTOR (yellow), MAPK (red), Wnt (blue)). Light blue arrows represent protein binding interaction. Dark blue arrows represent an enzymatic substrate to product conversion. Green arrows indicate activation, and red T means repression.
See this image and copyright information in PMC

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