title: Schwannomatosis: the overlooked neurofibromatosis?
abstract: OBJECTIVE
  Schwannomas are typically benign tumors that occur sporadically, in neurofibromatosis type 2 (NF2), or in an entity called "schwannomatosis." Schwannomatosis patients develop multiple schwannomas without involvement of the vestibular apparatus. Geneticists, neurologists, and pathologists have recognized that schwannomatosis is distinct from NF2, but schwannomatosis remains unfamiliar to many radiologists. This article reviews the current medical literature, highlighting the similarities and differences between the schwannomatosis and NF2 phenotypes, genotypes, clinical manifestations, management considerations, and imaging findings.
  
  
  CONCLUSIONS
  Imaging plays a critical role in diagnosing schwannomatosis, and a basic understanding of this syndrome is of interest to diagnostic radiologists. Moreover, it is imperative that radiologists be able to differentiate schwannomatosis from NF2 on imaging because there are significant differences in the management of these two diseases and clinical outcomes for affected patients.
authors.affiliation: Department of Radiology and Imaging Sciences, Indiana University School of Medicine, 550 University Blvd, Rm 06.3, Indianapolis, IN 46202-5253, USA. nakoontz@iupui.edu
authors.forename: Nicholas A
authors.initials: NA
authors.lastname: Koontz
authors.forename: Andrea L
authors.initials: AL
authors.lastname: Wiens
authors.forename: Atul
authors.initials: A
authors.lastname: Agarwal
authors.forename: Cynthia M
authors.initials: CM
authors.lastname: Hingtgen
authors.forename: Robert E
authors.initials: RE
authors.lastname: Emerson
authors.forename: Kristine M
authors.initials: KM
authors.lastname: Mosier
mesh_terms: D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans; D009442:Neurilemmoma / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D017253:Neurofibromatoses / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D010641:Phenotype; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy