Orbital Neurogenic Tumors: An Eye Care Service Experience in Turkey

Abstract

Introduction:This study aimed to evaluate the clinical and pathological features and treatment outcomes of neurogenic tumors developing in the orbit.
 Materials and Methods: A retrospective study was conducted on the medical records of 23 patients diagnosed with orbital neurogenic tumors between 2008 and 2020 in the ophthalmology clinic. Clinicopathologic features and treatment results of this patient group were evaluated.
 Results: Twenty-three patients, mean (SD) age 33.34±20.18 (min-max 4-60) years, were included in this study. Median follow-up time was 56.7±42.7 (min-max 9-120) months. At the time of presentation, 15 (65%) patients had proptosis, 8 (35%) patients had strabismus and reduced vision. Histopathologic diagnosis was made after lateral orbitotomy through the skin in 16 patients (69.5%) and medial orbitotomy through the conjunctiva in 4 patients (17.4%). The diagnosis was established clinically in three (13%) cases. Ten patients (43.4%) underwent whole excisional biopsy, nine patients (391.1%) underwent incisional biopsy, and one patient (4.3%) underwent subtotal excisional biopsy. Nine (39.1%) of the orbital neurogenic tumors were diagnosed as meningiomas based on histological and clinical findings, eight (34.8%) as optic nerve gliomas, and the other six (26%) as peripheral nerve origin tumors. Four (44.5%) of the meningiomas originated from the sphenoid wing, and five (55.5%) from the optic nerve sheath. As a treatment modality, external radiotherapy was administered to fifteen patients (65.2%), cyberknife radiosurgery to one patient (4.3%), chemotherapy to one patient (4.3%), and exenteration surgery to one patient (4.3%).
 Discussion: According to our study, meningioma, optic nerve glioma, and peripheral nerve sheath tumors were the most frequent neurogenic tumors of the orbit. Gliomas and meningiomas of the optic nerve sheath were treated with external radiation. Total excision was performed for schwannoma and solitary neurofibroma among the peripheral nerve sheath tumors, while subtotal excision was performed for infiltrative plexiform neurofibroma. With the treatments applied, survival and the visual prognosis were satisfactory.