- Authors:
- Karlyne M Reilly,
- AeRang Kim,
- Jaishri Blakely,
- Rosalie E Ferner,
- David H Gutmann,
- Eric Legius,
- Markku M Miettinen,
- R Lor Randall,
- Nancy Ratner,
- N L Jumbé,
- Annette Bakker,
- David Viskochil,
- Brigitte C Widemann,
- Douglas R Stewart
J Natl Cancer Inst 2017 Aug;109(8)
Rare Tumor Initiative, Laboratory of Pathology, and Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD; Center for Cancer and Blood Disorders, Children's National Medical Center, Washington, DC; Department of Neurology, Johns Hopkins Hospital, Baltimore, MD; Neurofibromatosis Center, Department of Neurology Guy's Hospital London, London, UK; Department of Neurology, Washington University School of Medicine, St. Louis, MO; Department of Human Genetics, University of Leuven, Leuven, Belgium; Hunstman Cancer Institute, University of Utah, Salt Lake City, UT; Division of Experimental Hematology and Cancer Biology, Department of Pediatrics, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH; Bill and Melinda Gates Foundation, Seattle, WA; Children's Tumor Foundation, New York, NY; Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD.
Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma for which the only effective therapy is surgery. In 2016, an international meeting entitled "MPNST State of the Science: Outlining a Research Agenda for the Future" was convened to establish short- and long-term research priorities. Key recommendations included the: 1) development of standardized, cost-efficient fluorodeoxyglucose positron emission tomography and whole-body magnetic resonance imaging guidelines to evaluate masses concerning for MPNST; 2) development of better understanding and histologic criteria for the transformation of a plexiform neurofibroma to MPNST; 3) establishment of a centralized database to collect genetic, genomic, histologic, immunohistochemical, molecular, radiographic, treatment, and related clinical data from MPNST subspecialty centers in a standardized manner; 4) creation of accurate mouse models to study the plexiform neurofibroma-to-MPNST transition, MPNST metastasis, and drug resistance; 5) use of trial designs that minimize regulatory requirements, maximize availability to patients, consider novel secondary end points, and study patients with newly diagnosed disease.
Read More