The management of symptomatic hyperostotic bilateral spheno-orbital meningiomas: patient series

Abstract

Background: The occurrence of hyperostotic bilateral spheno-orbital meningiomas (BSOMs) is very rare. Patients present with bilateral symptoms and require bilateral treatment. This series describes 6 patients presenting to 2 UK neurosurgical units and includes a literature review. To the best of the authors' knowledge, this is the largest series documented.

Observations: This is a retrospective review of patients with BSOMs presenting between 2006 and 2023. Six females, whose mean age was 43 (range: 36-64) years, presented with features of visual disturbance. Bilateral sphen-oorbital meningiomas were identified. All patients underwent bilateral staged resections. The patients had an initial improvement in their symptoms. Extensive genetic testing was performed in 4 patients, with no variants in the NF2, LZTR1, SMARCB1, SMARCE1, and SMARCA4 genes or other variants detected. The mean follow-up was 100.3 (range: 64-186) months. Sixty-seven percent of patients had good long-term visual acuity. The progression rate was 75% and was particularly aggressive in 1 patient. Four patients required radiation therapy, and 2 needed further surgery.

Lessons: Hyperostotic BSOMs are extensive, challenging tumors causing significant disability. They can recur, with significant patient impact. Multidisciplinary management and indefinite long-term follow-up are essential. The biology of these tumors remains unclear. As molecular testing expands, the understanding of BSOM oncogenesis and potential therapeutic targets is likely to improve.

Keywords: genetics; management; meningioma; multidisciplinary; neurosurgery; spheno-orbital.

FIG. 1

Case 1. Contrast T1-weighted magnetic resonance imaging (MRI) scans. A: Preoperative imaging showing the right spheno-orbital meningioma (SOM) enhancing homogeneously and extending into the right orbit. B: Postoperative scan obtained after a right-sided operation. The extent of excision can be seen on the right side. The growing SOM on the left side is clearly identified compared to the previous scan, with extension into the orbit. C: The most recent scan showing bilateral resection. Reconstruction mesh can be identified on the left side.

FIG. 2

Case 2. Preoperative imaging. A: Computed tomography (CT) head scan showing extensive hyperostosis at the skull base. B: Contrast T1-weighted MRI scan showing the soft-tissue component and compression of the optic nerves. C: Contrast T1-weighted MRI scan showing other abnormalities in the pineal and posterior parasagittal regions.

FIG. 3

Case 3. Contrast T1-weighted MRI scans. A: Preoperative imaging. The left SOM is identified with involvement of the lateral wall of the orbit, cavernous sinus, optic foramen, and posterior ethmoid region. There is no right-sided lesion. B: Eighty-six-month follow-up scan showing the growing right SOM extending into the superior orbital fissure and cavernous sinus, with an extraosseous soft-tissue component in the lateral orbit and middle fossa. C: Most recent scan showing the bilateral extent of resection.

FIG. 4

Case 6. Contrast T1-weighted MRI scans with CT. A: Preoperative imaging. The left SOM was identified, involving the lateral sphenoid wing and the roof, floor, and lateral wall of the orbit, with extensive hyperostosis along the lateral orbital wall, orbital roof, and temporal bone. There was a suspicion of dural enhancement along the right greater wing of the sphenoid, but no tumor was confidently identified. B: Postoperative 93-month follow-up scans showing the growing right SOM. Sphenoid and lateral orbital wall hyperostosis can be identified on the right side. Reconstruction material as well as residual hyperostosis can be seen on the left side. C: Most recent scan showing the bilateral extent of resection.