Malignant peripheral nerve sheath tumor: models, biology, and translation

Bandarigoda N. Somatilaka, Ali Sadek, Renee M. McKay, Lu Q. Le

Research output: Contribution to journalReview articlepeer-review

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients with Neurofibromatosis Type 1. They are associated with poor prognosis and are the leading cause of mortality in NF1 patients. MPNSTs can also develop sporadically or following exposure to radiation. There is currently no effective targeted therapy to treat MPNSTs and surgical removal remains the mainstay treatment. Unfortunately, surgery is not always possible due to the size and location of the tumor, thus, a better understanding of MPNST initiation and development is required to design novel therapeutics. Here, we provide an overview of MPNST biology and genetics, discuss findings regarding the developmental origin of MPNST, and summarize the various model systems employed to study MPNST. Finally, we discuss current management strategies for MPNST, as well as recent developments in translating basic research findings into potential therapies.

Original languageEnglish (US)
Pages (from-to)2405-2421
Number of pages17
JournalOncogene
Volume41
Issue number17
DOIs
StatePublished - Apr 22 2022

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Cancer Research

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