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Sarcoma
Volume 2017, Article ID 7429697, 10 pages
https://doi.org/10.1155/2017/7429697
Review Article

Malignant Peripheral Nerve Sheath Tumors State of the Science: Leveraging Clinical and Biological Insights into Effective Therapies

1Center for Cancer and Blood Disorders, Children’s National Health System, 111 Michigan Ave NW, Washington, DC 20010, USA
2Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, 9609 Medical Center Drive, Room 6E450, Bethesda, MD 20892, USA
3Rare Tumors Initiative, OD, CCR, National Cancer Institute, 37 Convent Drive, Bethesda, MD 20814, USA
4University of Utah, 295 Chipeta Way, Salt Lake City, UT 84108, USA
5Center for Cancer Research, National Cancer Institute, 10 Center Drive, Room 2S235C, Building 10, Bethesda, MD 20892, USA
6National Cancer Institute, Pediatric Oncology Branch, 10 Center Drive, Room 1-3742, Building 10, Bethesda, MD 20892, USA

Correspondence should be addressed to AeRang Kim; gro.lanoitansnerdlihc@mikea

Received 6 February 2017; Accepted 3 April 2017; Published 16 May 2017

Academic Editor: Alexander Lazar

Copyright © 2017 AeRang Kim et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and biology of MPNST through imaging and genomic advances since 2002. Genetically engineered mouse models that develop MPNST spontaneously have greatly facilitated preclinical evaluation of novel drugs for translation into clinical trials led by consortia efforts. Continued work in identifying alterations that contribute to the transformation, progression, and metastasis of MPNST coupled with longitudinal follow-up, biobanking, and data sharing is needed to develop prognostic biomarkers and effective prevention and therapeutic strategies for MPNST.