Neurofibromatosis Type 2: Optic Nerve Sheath Meningioma in one Orbit, Intramuscular Schwannoma in the Other


KIRATLI H. , Yildiz S. , Soylemezoglu F.

ORBIT-AN INTERNATIONAL JOURNAL ON ORBITAL DISORDERS AND FACIAL RECONSTRUCTIVE SURGERY, vol.27, no.6, pp.451-454, 2008 (Journal Indexed in ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 27 Issue: 6
  • Publication Date: 2008
  • Doi Number: 10.1080/01676830802350356
  • Title of Journal : ORBIT-AN INTERNATIONAL JOURNAL ON ORBITAL DISORDERS AND FACIAL RECONSTRUCTIVE SURGERY
  • Page Numbers: pp.451-454

Abstract

A highly unusual patient with neurofibromatosis type 2 (NF2) presenting with simultaneous bilateral orbital tumors is described. A12-year-old girl with a family history of NF2 was examined because of bilateral proptosis. Visual acuities were light perception RE and 20/40 LE. Magnetic resonance imaging studies showed bilateral cerebellopontine angle tumors, a tumor surrounding the right intraorbital optic nerve, and a large left lateral orbital mass mixed with the lateral rectus muscle. The histopathological diagnoses following incisional biopsies were right optic nerve sheath meningioma and left intramuscular schwannoma. The left-sided orbital schwannoma and the right-sided vestibular schwannoma were treated with fractionated stereotactic radiotherapy. This patient enlarges the spectrum of clinical presentations that can be encountered at young age in patients with NF2.