Neurofibromatosis type 2 with multiple plexiform schwannomas.

Abstract

Multiple plexiform schwannomas (PS) or neurilemmomas are very rare nerve sheath tumors, showing multiple schwannomas of the skin and other soft tissues, brain, or spinal cord with histopathologic characteristic of interwoven Antonio-A type tissues(1) but without other signs of neurofibromatosis type 2 (NF2). Neurofibromatosis type 2 is much less common than neurofibromatosis type 1 (NF1), with a symptomatic prevalence of 1 in 210,000,(2) and, to the authors' knowledge, only six cases of NF2 with multiple plexiform schwannomas have been reported in the literature.(3-5) We report a rare case of multiple cutaneous plexiform schwannomas associated with characteristic features of NF2 including bilateral acoustic neurilemomas and intracranial meningioma.