A pediatric case of neurofibromatosis type 2 presenting as a plexiform schwannoma of the intraparotid facial nerve: A review of the world literature on the presenting pathology

Summary We report a case of a 12-year-old boy who presented with a 1-year history of left facial swelling. During surgical exploration of the parotid gland, a plexiform schwannoma of the facial nerve was discovered. Subsequently other features of neurofibromatosis type 2 (NF2) were unravelled—bilateral intracanalicular facial schwannomas, and ependymoma of the spine. We detail the course that led to achieving this extremely rare association. This is the eighth case of plexiform schwannoma associated with neurofibromatosis type 2 reported to date, the first ever to involve a cranial nerve, and the third to present in the pediatric population.