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

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.

Mosaic Neurofibromatosis Type 1: A Systematic Review

“…Three hundred twenty individuals were identified from the case reports and two large case series, and we present them separately. Table shows the 157 well‐detailed individual cases from the 109 articles , Table shows the 124 individuals that Ruggieri and Huson reported, and Table shows the 39 that Listernick et al described.…”