Orbito-cranial schwannoma – A multicenter experience

Yinon Shapira, Valerie Juniat1, Tarjani Dave, Ahsen Hussain, Daniel McNeely, Akihide Watanabe, Akiko Yoneda5, Peerooz Saeed, Kyung In Woo, Thomas Hardy, Dinesh Selva

Meeting:  2022 RANZCO


Date:      -

Session Title: FREE PAPERS – Oculoplastic/Ocular Oncology

Session Time:      -

Purpose: To describe the features, management approaches and outcomes of orbito-cranial schwannomas.

Methods: Retrospective review of 10 patients with orbito-cranial schwannomas managed in six orbital services over 22 years. Data collected included demo- graphics, presenting features, neuroimaging characteristics, histology, management approach, complications and outcomes.

Results: Mean age of the patients was 41.4 ± 19.9 years and six (60%) were females. The majority presented with proptosis (90%), limited extraocular motility (80%), eye- lid swelling (60%) and optic neuropathy (60%). Most lesions (80%) involved the entire anterior-posterior span of the orbit, with both intra- and extraconal involve- ment. All tumours involved the orbital apex, the supe- rior orbital fissure and extended at least to the cavernous sinus. Surgical resection was performed for all. Seven (70%) were completely or subtotally resected combining an intracapsular approach by an orbital- neurosurgical collaboration, with no recurrence on post- operative follow-up (6-186 months). Three underwent tumour debulking. Of these, two remained stable on follow-up (6-34 months) and one showed progression of the residual tumour over nine years (cellular schwannoma on histology) necessitating stereotactic radiotherapy (SRT) for local control. Adjuncts to the orbito-cranial resection included peri-operative frozen
section (n = 5), endoscopic transorbital approach
(n = 2) and image-guided navigation (n = 1). Post- surgical adjuvant SRT was used in three subjects.

Conclusions: The possibility of successful surgical control in complex orbito-cranial schwannomas is highlighted. A combined neurosurgical/orbital approach with consider- ation of an intracapsular resection is recommended. Recurrence may not occur with subtotal excision and observation may be reasonable. Adjunctive SRT for pro- gression or residual tumour can be considered.