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Rabih Hage,1 Claire Alapetite,2 Hervé Brisse,3,4 Kevin Zuber,5 Augustin Lecler,6 Guillaume Lot,7 Caroline Le Guerinel,7 Catherine Vignal-Clermont,1 Herve Boissonnet7 1Neuro-ophthalmology Department, Hôpital Fondation A. de Rothschild, Paris, France; 2Radiation Oncology Department, and Proton Center Institut Curie, Paris-Orsay, France; 3Imaging Department, Institut Curie, Paris, France; 4Université des Sciences et Lettres, Paris, France; 5Department of Statistics and Clinical Research, Hôpital Fondation A. de Rothschild, Paris, France; 6Radiology Department, Hôpital Fondation A. de Rothschild, Paris, France; 7Neurosurgery Department, Hôpital Fondation A. de Rothschild, Paris, FranceCorrespondence: Rabih Hage Tel/Fax +33148036322Email rhage@for.parisPurpose: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France.Methods: We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at each follow-up MRI examination.Results: Sixty patients (50 women, 10 men; mean age, 45.2± 11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up.Conclusion: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.Keywords: optic nerve sheath meningioma, ONSM, proton beam therapy, PBT, compressive optic neuropathy, brain tumor, visual loss, opto-ciliary shunt, radiation retinopathy, RON

Objectives: Juvenile nasopharyngeal angiofibroma often recurs if the tumor is large. This report is a long-term follow-up of these cases. It establishes the prognostic values of tumor extensions, analyzes the anatomic factors involved in recurrences, describes the spontaneous evolution of remnants based on a radiographic follow-up, and evaluates the pertinence of complex combined surgical approaches for invasive tumors and the value of complementary endoscopy.Study Design: Retrospective review of 44 cases treated between 1985 and 1996.Methods: Statistical analysis of the correlation between recurrence and tumor extension as evaluated by systematic analysis of 18 putative tumor extensions on preoperative computed tomography scans.Results: Invasion of the skull base affected two-thirds of the patients, and the rate of recurrence was 27.5%. Extensions to the infratemporal fossa, sphenoid sinus, base of pterygoids and clivus, the cavernous sinus (medial), foramen lacerum, and anterior fossa were correlated with more frequent recurrence. Long-term radiographic follow-up revealed putative residual disease in nine asymptomatic patients: these remnants gradually involuted.Conclusions: The data in the current study emphasize the prognostic value of skull base invasion and the difficulty of complete resection of extended lesions. Tumor remnants detected in symptom-free patients should be kept under surveillance by repeated computed tomography scan, since involution may occur. Recurrent symptoms may be treated by radiotherapy (30 Gy) rather than by extended combined procedures. Endoscopic surgery should be combined with surgery for better control of skull base extensions. [ABSTRACT FROM AUTHOR]