What Are the Clinical, Imaging Differences Between Schwannomatosis and NF2?

Objective: To evaluate clinical and imaging features of schwannomatosis and highlight key differences from neurofibromatosis type 2 (NF2). Design: Retrospective review of published literature. Discussion: The peak incidence is reported to be between the ages of 30 and 60 years. Diagnosis requires exclusion of NF2 on clinical grounds and high-quality imaging of the vestibular nerves. To increase the specificity of the diagnosis of schwannomatosis, the revised criteria exclude all patients who fulfill the existing diagnostic criteria for NF2, have a first-degree relative with NF2, or who carry a known constitutional NF2 gene mutation. Patient age can serve as a useful discriminator; the diagnosis of schwannomatosis becomes more likely with increasing age. Other discriminators, such as concomitant meningiomas, gliomas, or neurofibromas, should sway the radiologist away from suggesting the diagnosis of schwannomatosis. Patients with schwannomatosis frequently present with pain, whereas N more...