Neurofibromatosis type 2: optic nerve sheath meningioma in one orbit, intramuscular schwannoma in the other.
A highly unusual patient with neurofibromatosis type 2 (NF2) presenting with simultaneous bilateral orbital tumors is described. A 12-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.
Kiratli
Hayyam
H
Department of Ophthalmology, Hacettepe University School of Medicine, Ankara, Turkey.
Yildiz
Sibel
S
Soylemezoğlu
Figen
F
D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D005260:Female; D007839:Functional Laterality; D006222:Hamartoma / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008577:Meningeal Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D008579:Meningioma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D019042:Muscle Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D009363:Neoplasm Proteins / Q000032:analysis; D016609:Neoplasms, Second Primary / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D009442:Neurilemmoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D016518:Neurofibromatosis 2 / Q000473:pathology* / Q000601:surgery; D009464:Neuroma, Acoustic / Q000737:chemistry / Q000473:pathology / Q000601:surgery; D009801:Oculomotor Muscles / Q000473:pathology; D009918:Orbital Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D016634:Radiosurgery; D020266:Radiotherapy, Conformal; D012164:Retinal Diseases / Q000473:pathology
D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins