Korean J Dermatol.  2005 Sep;43(9):1267-1271.

A Case of Multiple Cutaneous Plexiform Schwannomas Accompanying Neurologic Deficits

• Park ES
• Moon SE

Abstract

Plexiform schwannoma is a rare variant of benign schwannoma characterized by a multinodular and plexiform growth pattern. Schwannomatosis is a recently recognized disorder, defined as multiple, pathologically-proven schwannoma without vestibular tumors, diagnostic of neurofibromatosis 2. Although plexiform schwannoma is usually solitary, multiple plexiform schwannomas do also occur as an isolated finding or in association with neurofibromatosis 2. We describe a 7-year-old girl who had multiple cutaneous plexiform schwannomas and neurologic deficits such as sensorineural hearing loss and amblyopia on her left side. Brain magnetic resonance imaging and ophthalmologic examination was negative for vestibular schwannoma and eye stigmata of neurofibromatosis 2. However, as there is a possibility that the patient may eventually develop additional cranial tumors such as meningiomas or vestibular schwannomas, we therefore recommend that she is periodically screened.

Keyword

Multiple plexiform schwannoma; Sensorineural hearing loss

MeSH Terms

Amblyopia
Brain
Child
Christianity
Female
Hearing Loss, Sensorineural
Humans
Magnetic Resonance Imaging
Meningioma
Neurilemmoma*
Neurofibromatosis 2
Neurologic Manifestations*
Neuroma, Acoustic