Histopathologic Evaluation of Atypical Neurofibromatous Tumors and Their Transformation into Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis 1 Patients – A Consensus Overview

Summary Neurofibromatosis 1 (NF1) patients develop multiple neurofibromas, with 8-15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically challenging. In...

Full description

Saved in:
Bibliographic Details
Published in:Human pathology 2017-05, Vol.67, p.1-10
Main Authors: Miettinen, Markku M., MD, Antonescu, Cristina R., MD, Fletcher, Christopher D.M., MD, Kim, Aerang, MD, Lazar, Alexander J., MD, Quezado, Martha M., MD, Reilly, Karlyne M., PhD, Stemmer-Rachamimov, Anat, MD, Stewart, Douglas R., MD, Viskochil, David, MD, Widemann, Brigitte, MD, Perry, Arie, MD
Format: Article
Language:English
Subjects:
Pathology
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Summary Neurofibromatosis 1 (NF1) patients develop multiple neurofibromas, with 8-15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically challenging. In this overview, following a consensus meeting in October 2016, we outline the histopathologic features and molecular mechanisms involved in the malignant trans-formation of neurofibromas. Nuclear atypia alone is generally insignificant. However, with atypia, loss of neurofibroma architecture, high cellularity, and/or mitotic activity >1/50 but
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2017.05.010