Analysis of Clinical and Molecular Parameters in MPNST
Abstract
Malignant peripheral nerve sheath tumors (MPNSTs) are very rare cancers that carry a poor prognosis. About half occur in the context of neurofibromatosis type 1 (NF1), an autosomal dominant syndrome that affects approximately 1/3000 individuals world-wide. NF1 patients develop multiple slow-growing benign Schwann cell tumors (neurofibromas) throughout life, anywhere on the peripheral nervous system, from spinal nerve roots to nerve endings in the skin. Neurofibromas affecting larger nerves are called plexiform, which are thought to be congenital in origin and have an 8-13 percent risk of transformation to MPNST, which may not be detected until an existing tumor becomes painful. Because of MPNST rarity, there is no evidence-based treatment protocol. Rather, each institution manages cases uniquely, choosing from treatment modalities, with variable results. In addition, as the World Health Organization 2016 classification indicates, Clinically validated and reproducible grading systems for MPNST are generally lacking (Reuss et al., 2016), so neuropathologists have less-than optimal tools for diagnosis and prognosis. Complicating progress in MPNST clinical care is heterogeneity at several levels. Although most cases are seen in major medical centers, here are fewer than 20 retrospective studies in the literature, and these report somewhat different therapies, outcomes and prognostic factors.
Document Details
- Document Type
- Technical Report
- Publication Date
- Oct 01, 2021
- Accession Number
- AD1153342
Entities
People
- Anthony Yachnis
- Margaret R. Wallace
- Marybeth Horodyski
Organizations
- University of Florida