Douglas, Vivian Paraskevi; Douglas, Konstantinos A.A.; Cestari, Dean M.
Purpose of review
Optic nerve sheath meningiomas (ONSMs) are rare benign tumors of the anterior visual pathway which present with slowly progressive and painless vision loss and account for approximately 2% of all orbital tumors. This article provides an overview as well as an update on the ONSMs with regards to cause, epidemiology, clinical presentation, diagnosis, and management in adults and pediatric population.
The clinical presentation and prognosis of ONSMs can vary and largely depend on the location of tumor as well as the histologic type. Overall, the diagnosis is based on clinical presentation, examination, and neuroimaging findings. Nevertheless, delays in diagnosis or misdiagnosis are not uncommon and can result in higher morbidity rates. Recent advances in diagnostic as well as more effective and less-invasive treatment options are discussed in this review.
ONSMs are a rare cause of slowly progressive and inexorable visual loss. Although ONSM diagnosis depends on the characteristic clinical and radiologic findings, prompt diagnosis, and appropriate management is critical for favorable visual outcomes. Thus, current focus is optimizing diagnostic as well-treatment methods for patients with ONSMs.
Hiroyuki Sasano, Keigo Shikishima, Manabu Aoki, Tsutomu Sakai, Yuki Tsutsumi, Tadashi Nakano
The present study examined the efficacy and complications associated with intensity-modulated radiation therapy (IMRT) for optic nerve sheath meningioma (ONSM) in 15 cases and compared visual function before and after treatment.
Consecutively diagnosed patients with ONSM treated with IMRT were evaluated from 2012 to 2017. We categorized ONSM with three growth patterns (diffuse, fusiform, or globular). Visual acuity, visual fields, and optic disc findings were assessed before and after IMRT. Ocular and systemic complications were evaluated during and after treatment.
The 15 patients selected for analysis ranged in age from 33 to 77 years. Post-treatment observation periods were 8 to 57 months. After IMRT, tumor enlargement was not detected in any eyes, and tumor reduction was seen in 2 eyes. At final post-treatment follow-up, eyes with fusiform and globular growth maintained better visual acuity compared with pre-treatment, whereas 2 of 5 eyes with diffuse growth showed reduced vision. Five eyes with no apparent optic disc abnormality maintained better visual acuity compared with pre-treatment, whereas 8 of 10 eyes with disc edema and atrophy remained stable or showed reduced vision. Improvements were seen in all 5 eyes with optic discs negative for pre-treatment abnormalities. Final post-treatment visual field abnormalities improved in 11 eyes. All adverse events identified during IMRT improved rapidly during the treatment period.
IMRT for the treatment of ONSM achieved improvement and preserved visual function. In particular, early treatment with IMRT before the appearance of optic disc abnormalities can be more effective for improving visual function.
Ravi Pandit, Liliana Paris, Danielle S Rudich, Robert L Lesser, Mark J Kupersmith, Neil R Miller
Background/Aim Fractionated conformal radiotherapy (FCRT) is now used to treat vision-threatening optic nerve sheath meningioma (ONSM), but long-term efficacy and safety data are lacking; the purpose of this study was to assess these key data.
Methods This is a retrospective chart review with prospective follow-up of adult patients treated with FCRT for primary ONSM at four academic medical centres between 1995 and 2007 with ≥10 years of follow-up after treatment.
Results 16 patients were identified with a mean post-treatment follow-up of 14.6 years (range: 10.5–20.7 years). The mean age at symptom onset was 47.6 years (range: 36–60 years). FCRT was performed at a mean of 2.3 years after symptom onset (range: 0.2–14.0 years). At last follow-up, visual acuity had improved or stabilised in 14 of the 16 (88%) patients, and 11 (69%) had retained or achieved ≥20/40. The mean deviation on automated perimetry remained stable (−14.5 dB pretreatment vs −12.2 dB at last follow-up; p=0.68, n=10). Two (11%) patients had persistent pain, proptosis or diplopia, compared with six (38%) pretreatment (p=0.11). Two (13%) patients developed radiation retinopathy more than 6 months after completion of therapy, one (50%) of whom had worse visual acuity compared with pretreatment. No patient developed tumour involvement or radiation damage in the fellow eye.
Conclusion FCRT stabilises or improves visual function in patients with primary ONSM and is associated with a low risk of significant ocular sequelae. This treatment should be considered instead of surgery in patients with primary ONSM who require intervention due to loss of visual sensory and/or ocular motor function.
Cesare Zoia, Daniele Bongetta, Fabio Pagella, Elena R. Antoniazzi, Paolo Gaetani
However rare they may be, optic nerve sheath meningiomas are lesions that represent one-third of all intrinsic tumors of the optic nerve.1 When untreated, they can lead to blindness: Their surgical treatment, which is likewise a blinding procedure in most cases, is reserved for patients with exophtalmus.1 Another indication for surgery is to prevent their chiasmatic spread to the controlateral optic nerve.2 In recent years, endoscopic transnasal techniques have evolved to such an extent that now even the orbit can be reached with these types of approaches.
Daniel S Narayan, Ghislaine L Traber, Edwin Figueira, Adnan Pirbhai, Klara Landau, Garry Davis, John Crompton, Dinesh Selva
Purpose To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM).
Methods Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed.
Results The mean age at presentation was 11 years (range: 6–17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71–297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up.
Conclusions This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.
Anna Parlin, Alina Dumitrescu, William T. Nunery, Peter J. Timoney & Jason A. Sokol
Optic nerve sheath meningiomas (ONSM) and intra-conal orbital lymphomas are common entities on the differential of a retrobulbar optic nerve involving space-occupying lesion. In this study, we compare the pre-surgical diagnosis, based on clinical presentation and neuroimaging, to the surgical pathology results of intra-conal orbital lymphomas and ONSM. This is an IRB approved retrospective chart review of orbital lymphomas and optic nerve sheath meningiomas biopsied by a single surgeon over a 4-year period at a single institution. Pre-surgical diagnosis and surgical pathology were compared. Fifteen cases of orbital lymphoma were identified. Fourteen were excluded based on extra-conal location. The single histologically confirmed intra-conal orbital lymphoma had a pre-surgical diagnosis of ONSM. Four cases of optic nerve sheath meningioma were identified. Three of the 4 cases of histologically confirmed ONSM had a pre-surgical diagnosis of ONSM. One of the 4 had a pre-surgical diagnosis of lymphoma. Diagnosis based on surgical pathology differed from the pre-surgical diagnosis in 2 out of 5 cases showing that clinical diagnosis does not always correlate with histologic diagnosis. Although both diseases are typically managed with radiation therapy, the treatment dosage and systemic disease implications are very different. These findings emphasis the importance of biopsy in the diagnosis of orbital lesions surrounding the optic nerve.