Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with …
MM Miettinen, CR Antonescu, CDM Fletcher, A Kim… - Human pathology, 2017 - Elsevier
Summary Patients with neurofibromatosis 1 (NF1) develop multiple neurofibromas, with 8%
to 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, after a consensus meeting in October 2016,
we outline the histopathologic features and molecular mechanisms involved in the
malignant transformation of neurofibromas. Nuclear atypia alone is generally insignificant …
to 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, after a consensus meeting in October 2016,
we outline the histopathologic features and molecular mechanisms involved in the
malignant transformation of neurofibromas. Nuclear atypia alone is generally insignificant …
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