Forme Fruste of von Recklinghausen's Disease: Unilateral Association of an Orbital Neurofibroma, a Trigeminal Neurinoma, and an Acoustic Neurinoma

Author:

Kasuya Hidetoshi1,Kadowaki Hirotaka1,Yato Seiji1,Kubo Osami1,Kagawa Mizuo1,Kitamura Koichi1

Affiliation:

1. Department of Neurosurgery, Isesaki General Health Center Hospital, Gumma, and Department of Neurosurgery, Neurological Institute, Tokyo Women's Medical College, Tokyo, Japan

Abstract

Abstract A patient who presented with a tumor of the left ambient cistern, a left cerebellopontine angle tumor, and a left orbital tumor causing left hearing loss and left exophthalmos without café au lait spots or cutaneous neurofibromas is described. There was no family history of von Recklinghausen's disease. A cerebellopontine angle tumor removed by a suboccipital craniectomy was an acoustic neurinoma. An ambient cistern tumor was approached through a subtemporal route. A tumor arising from the trigeminal nerve was also a neurinoma. An orbital neurofibroma was excised by a frontal craniotomy with removal of the orbital roof. This rare unilateral association of neurinomas and a neurofibroma on the left side was thought to be a forme fruste of von Recklinghausen's disease, and it could be considered a presentation of a mosaic of von Recklinghausen's disease.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Clinical Neurology,Surgery

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