Malignant peripheral nerve sheath tumors: Prognostic and diagnostic markers and therapeutic targets

Holly Meany, Brigitte C. Widemann, Nancy Ratner

Research output: Chapter in Book/Report/Conference proceedingChapter

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 languageEnglish (US)
Title of host publicationNeurofibromatosis Type 1
Subtitle of host publicationMolecular and Cellular Biology
PublisherSpringer-Verlag Berlin Heidelberg
Pages445-467
Number of pages23
Volume9783642328640
ISBN (Electronic)9783642328640
ISBN (Print)3642328636, 9783642328633
DOIs
StatePublished - Aug 1 2012

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

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