Chapter 16 - Approach to patients with the neoplasms associated with neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis

Abstract

Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis (SWN) are rare autosomal dominant tumor suppressor conditions that predispose to tumors throughout the central and peripheral nervous systems. These syndromes present with characteristic tumors and often a constellation of skin and systemic findings that facilitate a clinical diagnosis, require close monitoring and appropriate surveillance, and when treatment is indicated, a multidisciplinary approach to management. In this chapter, we present a series of cases that highlight some of the most common clinical scenarios encountered in the care of people with NF1, NF2, and SWN including management of symptomatic and growing plexiform neurofibroma in an adult with NF1, optic pathway glioma in a child with NF1, non-optic pathway glioma in a child with NF1, bilateral vestibular schwannoma with variable hearing loss in a young adult with NF2, and the diagnosis and management of painful tumors in adults with SWN.