Walsh & Hoyt: Neurofibromatosis Type 2

NF2 is much less common than NF1, affecting 1:35,000 to 1:50,000 persons. CNS tumors are the major feature in NF2. Meningiomas, gliomas, and schwannomas are common, but bilateral vestibular schwannomas are nearly always present. In general, there are two categories of patients with NF2: those with an early-onset, rapid course associated with multiple other tumors in addition to bilateral vestibular schwannomas, and those with a late-onset, more benign course and only bilateral vestibular schwannomas.