6 Neurofibromas and Malignant Peripheral Nerve Sheath Tumors (MPNST) in Neurofibromatosis 1 The growth of multiple neurofibromas is not only the hallmark feature of neurofibromatosis 1 (NF1), but also is…

9 Other Features of Neurofibromatosis 1 In addition to the common manifestations of neurofibromatosis 1 (NF1), a variety of seemingly unrelated manifestations are associated with the disorder. These less common…

2 The Many Faces of Neurofibromatosis The diagnosis of neurofibromatosis is based on clinical criteria established mainly through a detailed medical history and physical examination. Although other varieties of neurofibromatosis…

3 The Pathogenesis of Neurofibromatosis 1 and Neurofibromatosis 2 The neurofibromatoses are genetic disorders. NF1 and NF2 are each caused by a mutation in a known specific gene. The quest…

10 Diagnosis and OverallManagement ofNeurofibromatosis 2 The hallmark feature of neurofibromatosis 2 (NF2) is a slow-growing tumor on the eighth cranial nerve in both ears. This nerve has two branches….

12 Schwannomatosis Schwannomatosis is a newly recognized form of neurofibromatosis that is distinct from neurofibromatosis 1 (NF1) and neurofibromatosis 2 (NF2), in terms of both its primary clinical features and…

11 Management of Particular Neurofibromatosis 2 Features A diagnosis of confirmed or probable neurofibromatosis 2 (NF2) raises a host of new challenges. Optimal management of the disorder depends on several…