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Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect

1
Sarcoma Services, Department of Orthopaedics, Huntsman Cancer Institute and Primary Childrens Medical Center, University of Utah, Salt Lake City, UT 84112, USA
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Department of Pathology, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112, USA
3
Division of Medical Genetics, Department of Pediatrics, University of Utah, Salt Lake City, UT 84112, USA
*
Authors to whom correspondence should be addressed.
Cancers 2013, 5(2), 519-528; https://doi.org/10.3390/cancers5020519
Received: 4 March 2013 / Revised: 9 April 2013 / Accepted: 3 May 2013 / Published: 8 May 2013
(This article belongs to the Special Issue Soft Tissue and Bone Sarcoma)
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. View Full-Text
Keywords: MPNST; neurofibromatosis; nerve sheath tumor; atypical neurofibroma; low grade MPNST MPNST; neurofibromatosis; nerve sheath tumor; atypical neurofibroma; low grade MPNST
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Bernthal, N.M.; Jones, K.B.; Monument, M.J.; Liu, T.; Viskochil, D.; Randall, R.L. Lost in Translation: Ambiguity in Nerve Sheath Tumor Nomenclature and Its Resultant Treatment Effect. Cancers 2013, 5, 519-528.

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