NF2-Related Tumors and Malignant Peripheral Nerve Sheath Tumors

Neurofibromatosis (NF) types 1 and 2 are classified as phacomatoses with an autosomal-dominant inheritance pattern. They are important in the field of neuro-oncology as they predispose affected patients to a number of CNS tumors including acoustic schwannoma, meningioma, glioma, ependymoma, and malignant peripheral nerve sheath tumor (MPNST). The management of tumors in patients with NF can differ from that in the non-NF population owing to a natural history that may be more aggressive, a greater need to preserve function in patients with acoustic schwannoma, and the concern for increased risk of radiation-induced tumors in this population. The management of NF-related acoustic schwannoma, meningioma, and MPNST is discussed here, while the management of glioma and ependymoma is presented in separate chapters.