title: A case of multiple schwannomas of the trigeminal nerves, acoustic nerves, lower cranial nerves, brachial plexuses and spinal canal: schwannomatosis or neurofibromatosis?
abstract: In most cases, while schwannoma is sporadically manifested as a single benign neoplasm, the presence of multiple schwannomas in one patient is usually indicative of neurofibromatosis 2. However, several recent reports have suggested that schwannomatosis itself may also be a distinct clinical entity. This study examines an extremely rare case of probable schwannomatosis associated with intracranial, intraspinal and peripheral involvements. A 63-year-old woman presented with a seven-year history of palpable lumps on both sides of the supraclavicular area and hearing impairment in both ears. On physical examination, no skin manifestations were evident. Facial sensory change, deafness in the left ear and decreased gag reflex were revealed by neurological examination. Magnetic resonance imaging revealed multiple lesions of the trigeminal nerves, acoustic nerves, lower cranial nerves, spinal accessory nerve, brachial plexuses, and spinal nerves. Pathological examination of tumors from the bilateral brachial plexuses, the spinal nerve in the T8 spinal position and the neck mass revealed benign schwannomas. Following is this patient case report of multiple schwannomas presenting with no skin manifestations of neurofibromatosis.
authors.affiliation: Department of Neurosurgery, Pundang CHA Hospital, Pochon CHA University, Sungnam, 463-070, Korea. jyahn@cha.ac.kr
authors.forename: Jung Yong
authors.initials: JY
authors.lastname: Ahn
authors.forename: Seong Oh
authors.initials: SO
authors.lastname: Kwon
authors.forename: Moon Soo
authors.initials: MS
authors.lastname: Shin
authors.forename: Jeong Yun
authors.initials: JY
authors.lastname: Shim
authors.forename: Ok Joon
authors.initials: OJ
authors.lastname: Kim
mesh_terms: D020516:Brachial Plexus Neuropathies / Q000473:pathology* / Q000601:surgery; D003390:Cranial Nerve Neoplasms / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009442:Neurilemmoma / Q000473:pathology* / Q000601:surgery; D017253:Neurofibromatoses / Q000473:pathology*; D013115:Spinal Canal / Q000473:pathology*; D020433:Trigeminal Nerve Diseases / Q000473:pathology* / Q000601:surgery; D000160:Vestibulocochlear Nerve Diseases / Q000473:pathology* / Q000601:surgery