Abstract
There is much ambiguity surrounding the diagnosis of nerve sheath tumors, including atypical neurofibroma and low-grade MPNST, and yet, the distinction between these entities designates either benign or malignant behavior and thus carries presumed profound prognostic importance that often guides treatment. This study reviews the diagnostic criteria used to designate atypical neurofibroma from low-grade MPNSTs and reviews existing literature the natural history of each of these tumors to see if the distinction is, in fact, of importance.
Highlights
Peripheral nerve sheath tumors (PNSTs) comprise a spectrum of neoplastic potential ranging from benign neurofibromas and schwannomas to high-grade malignant peripheral nerve sheath tumors (MPNSTs)
Atypical neurofibromas and low-grade MPNSTs reside in an undefined, nebulous middle ground that presents a treatment challenge to physicians and surgeons
We review the present literature on atypical neurofibromas and low-grade
Summary
Peripheral nerve sheath tumors (PNSTs) comprise a spectrum of neoplastic potential ranging from benign neurofibromas and schwannomas to high-grade malignant peripheral nerve sheath tumors (MPNSTs). High-grade MPNSTs are fulminantly malignant lesions with clinical outcomes on par with the worst of soft tissue sarcomas, despite aggressive treatment. Along this spectrum, atypical neurofibromas and low-grade MPNSTs reside in an undefined, nebulous middle ground that presents a treatment challenge to physicians and surgeons. While convention defines atypical neurofibromas as benign lesions and low-grade MPNSTs as malignant, this distinction may not be so clear. In this analysis, we review the present literature on atypical neurofibromas and low-grade
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