Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are the most common malignancy associated with NF1. At present, complete surgical removal is the only successful treatment for MPNST, and the outcome for unresectable, recurrent, or metastatic MPNST remains poor. Because 50 % of MPNSTs arise in individuals with NF1, and because the prognosis of NF1-associated MPNST appears to be worse than that of sporadic tumors, many studies of MPNST have used NF1 models and comparisons between sporadic and NF1 MPNST. Currently, molecular analyses and preclinical testing are beginning to aid in the identification of promising therapies to target these neoplasms. Knowledge of the epidemiology, clinical presentation, diagnosis, and prognostic factors for MPNST is anticipated to allow for earlier detection of, and more successful treatment for, MPNST.
Original language | English (US) |
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Title of host publication | Neurofibromatosis Type 1 |
Subtitle of host publication | Molecular and Cellular Biology |
Publisher | Springer-Verlag Berlin Heidelberg |
Pages | 445-467 |
Number of pages | 23 |
Volume | 9783642328640 |
ISBN (Electronic) | 9783642328640 |
ISBN (Print) | 3642328636, 9783642328633 |
DOIs | |
State | Published - Aug 1 2012 |
Externally published | Yes |
ASJC Scopus subject areas
- Medicine(all)
- Biochemistry, Genetics and Molecular Biology(all)