Advance Access: Malignant Peripheral Nerve Sheath Tumors are not a Feature of Neurofibromatosis Type 2 in the Unirradiated Patient

Neurofibromatosis type 2 (NF2) is an autosomal dominant inherited condition characterized by the development of bilateral vestibular schwannomas (VS), but also a variety of other tumors both in the central and peripheral nervous system. This includes other cranial, spinal, peripheral nerve and intradermal schwannomas, cranial and spinal meningiomas, and intrinsic tumors of the spine and brain stem, which are predominantly ependymomas. These tumors are categorized in the Manchester criteria1 (Table). Two recent reviews have highlighted a potential increased risk of spontaneous malignant peripheral nerve sheath tumors (MPNST) in NF2.2,3 We have interrogated the Manchester NF2 database of 1253 NF2 patients for the presence of histologically proven MPNST and reviewed the literature.

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