Spheno-Orbital Meningioma With an Orbital Cystic Component.

Anaplastic spheno-orbital meningioma: rapid growth after extensive exenteration

Spheno-orbital meningiomas are mainly defined as primary en plaque tumors of the lesser and greater sphenoid wings, invading the underlying bone and adjacent anatomical structures. The patients, mostly women in their fifties, generally present with a progressive, unilateral, and nonpulsatile proptosis, often associated with cosmetic deformity and optic nerve damage. Surgical resection is currently the gold standard of treatment in case of optic neuropathy, significant symptoms, or radiological progression. The surgical strategy should take into account the morphology of the tumor, its epicenter at the level of the sphenoid wing, and the invasion of adjacent anatomical structures. Surgery stabilizes or improves visual function and oculomotricity in most cases but it is rare that the proptosis recovers completely. Gross total resection is hard to achieve considering the complex anatomy of the spheno-orbital region and the risk of inducing cranial nerve deficits. Rare cases of WHO grade II or III meningiomas warrant adjuvant radiotherapy. Tumor residues after subtotal resections of WHO grade I meningiomas are first radiologically monitored and then treated by stereotactic radiosurgery in case of progression.

(1) Background: Spheno-orbital meningioma (SOM) is a very rare subtype of meningioma which arises from the sphenoid ridge with an orbital extension. It exhibits intraosseous tumor growth with hyperostosis and a widespread soft-tissue growth at the dura. The intra-orbital invasion results in painless proptosis and slowly progressing visual impairment. (2) Methods: We present a case of a 46-year-old woman with SOM and compressive optic nerve neuropathy related to it. Her corrected distance visual acuity (CDVA) was decreased to 20/100, she had extensive visual field (VF) scotoma, dyschromatopsia, impaired pattern-reversal visual-evoked potential (PVEP), and decreased thicknesses of the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC), measured with the swept-source optical coherence tomography (SS-OCT), and a pale optic nerve disc in her left eye. Brain CT and MRI showed a lesion at the base of the anterior cranial fossa, involving the sphenoid wing and orbit. Pterional craniotomy and a partial removal of the tumor at the base of the skull and in the left orbit with the resection of the lesional dura mater and bony defect reconstruction were performed. (3) Results: The histological examination revealed meningothelial meningioma (WHO G1). Decreased CDVA and VF defects completely recovered, and the color vision score and PVEP improved following the surgery, but RNFL and GCC remained impaired. No tumor recurrence was observed at a follow-up of 78 months. (4) Conclusions: Optic nerve dysfunction has the capacity to improve once the compression has been relieved despite the presence of the structural features of optic nerve atrophy.

Neoplasms of the Nervous System and Related Topics

Objectives: The management of spheno-orbital meningiomas (SOM) is challenging due to complex characteristics of these tumours, such as extension into the orbit, hyperostosis of the greater sphenoid wing (GSW), and invasion of superior orbital fissure (SOF), cavernous sinus (CS), and optic canal. To address this challenge, this study reports the results of microsurgically treated SOM patients and the nuances that affect the surgical technique. Methods: The clinical data of 14 consecutive patients who underwent surgery through a microsurgical transcranial approach is collected retrospectively. Results: Among 14 patients, exophthalmos was present in all patients and facial numbness and visual acuity decline both in 50% of patients. Preoperative radiological imaging showed SOF narrowing in 7 cases, optic canal compression in 6 cases, and CS invasion in 5 cases. Our surgical technique relies on direct removal of affected GSW promoting the removal of temporal intradural tumor and decompression of lateral wall of the orbit, optic canal, and SOF. Total removal of GSW is found to be beneficial in treatment SOMs since tumor invasion to affected bone was met in all cases. Simpson grade 1 resection was achieved in 64% of cases. CS invasion present in 5 cases left intentionally unremoved. In two cases, second surgery was required, while in four cases, radiotherapy was necessary. Conclusions: Microsurgical treatment aiming for maximal safe resection with preservation of contents within the CS is the best option in the first-line treatment of SOMs. Aggressive removal of the bone tumor is beneficial in oncological control of disease.

To determine treatment outcomes, recurrence rates, and predictors of recurrence, to inform future therapeutic approaches for spheno-orbital meningiomas (SOM). A retrospective single-center study of SOM treated from 1990 to 2021 was conducted with comprehensive neuro-ophthalmologic follow-up at Columbia University Medical Center (CUMC). Recurrence requiring reintervention was defined clinically as worsening of visual acuity, visual field defect, or ocular motility after an initial period of stabilization or 6 months of improvement following treatment, or radiologically as either a regrowth with an increase in tumor size by 20% at the site of previous growth or a new region of tumor growth. In total 46 patients met the inclusion criteria. The mean follow-up was 106 months (range 1-303). Dictated by the phenotype of the disease, patients underwent either gross- (50%), near- (17%), or subtotal resection (26%). Removal of the anterior clinoid process (ACP) was performed in 52% of patients. Nine patients (20%) required an enucleation or exenteration. Radiotherapy was employed at some point of treatment in 50% of cases. Inherited cases (24%) were referred to CUMC for treatment following 1 or more recurrences. The total recurrence rate, including inherited cases, was 54%, occurring at a mean interval of 43 months. The recurrence rate of patients treated solely at CUMC was 40%, occurring at a mean interval of 41 months. A subset of patients (32%) had 2 or more recurrences. Histopathology at the first surgery was WHO grade I (87%) and II (13%) and at the final surgery was WHO grade I (74%), II (21%), and III (4%). A subset of grade I tumors that received radiotherapy (35%) evolved to a higher grade or developed multiple recurrences without a change in histologic grade I. Grade II tumors and treatment with radiotherapy increased the odds of recurrence. Removal of the ACP and gross total resection decreased the odds of recurrence. Due to the routinely long interval to tumor recurrence, lifelong surveillance of patients with SOM is prudent. ACP resection and gross total resection, where possible, reduce tumor recurrence and the need for further treatment. Radiotherapy should be reserved for higher-grade meningiomas and select grade I tumors.

Spheno-orbital meningiomas are complex tumours involving the sphenoid wing and orbit. Various surgical strategies are available but treatment remains challenging and patients often require more than one surgical procedure. This study evaluated whether smaller surgical approaches and newer reconstructive methods impacted the surgical and clinical outcomes of patients undergoing repeat surgery. We retrospectively analysed the medical records of consecutive patients who underwent surgery for a spheno-orbital meningioma at a single tertiary centre between 2005 and 2016. We recorded procedural details and analysed complications, postoperative visual status and patient-reported cosmetic outcome. Thirty-four procedures were performed in 31 patients (M:F 12:22, median age 49years) including 19 (56%) primary operations and 15 (44%) repeat procedures. Seven patients (20.5%) had a pterional craniotomy, 19 (56%) had a standard orbitozygomatic craniotomy and 8 (23.5%) underwent a modified mini-orbitozygomatic craniotomy. Calvarial reconstruction was required in 19 cases with a variety of techniques used including titanium mesh (63%), PEEK (26%) and split calvarial bone graft (5%). Total tumour resection (Simpson grade I-II) was significantly higher in patients undergoing primary surgery compared with those having repeat surgery (41% and 0%, respectively; p = 0.0036). Complications occurred in 14 cases (41%). Proptosis improved in all patients and visual acuity improved or remained stable in 93% of patients. Cosmetic outcome measures were obtained for 18 patients (1 = very poor; 5 = excellent): 1-2, 0%; 3, 33%; 4, 28%; 5, 39%. Tumour recurrence requiring further surgery occurred in four patients (12%). There was no significant difference in clinical outcomes between patients undergoing primary or repeat surgery. Spheno-orbital meningiomas are highly complex tumours. Surgical approaches should be tailored to the patient but good clinical and cosmetic outcomes may be achieved with a smaller craniotomy and custom-made implants, irrespective of whether the operation is the patient's first procedure.

Advances in surgical approaches have improved access for total or near-total resection of spheno-orbital meningiomas (SOM). Herein, the outcomes of multidisciplinary resection and reconstruction of SOM via frontotemporal orbitozygomatic craniotomy at a single institution are evaluated. A retrospective chart review was performed of all patients with SOM who underwent joint neurosurgical and oculofacial plastic resection via frontotemporal orbitozygomatic craniotomy between January 1999 and December 2018. Demographic data, clinical presentation, risk factors for meningioma, radiographic imaging, pathology results, postoperative outcomes, and surgical complications were reviewed. The outcome measures assessed included visual acuity, pupillary function, color vision, ocular motility, visual fields, and proptosis. A total of 48 patients were identified having had multidisciplinary frontotemporal orbitozygomatic craniotomy for SOM of which 43 met inclusion criteria. A mean follow-up period of 23.9 ± 20.4 months (range 1-60) was observed. There were 35 patients who underwent primary resection and 8 patients who had prior surgical resection. The main presenting complaints were proptosis (88%), headache (44%), and reduced vision (12%). Gross total resection was achieved in 15 patients (35%) while near-total or subtotal resection was achieved in the remainder. Histologic analysis revealed World Health Organization grade I meningioma in 72% of tumors, grade II in 23%, and grade III in 5%. Mean visual acuity (Logarithm of the Minimum Angle of Resolution) improved from 0.24 ± 0.46 preoperatively to 0.09 ± 0.13 postoperatively (p = 0.03). Surgery improved proptosis, with a reduction in mean Hertel exophthalmometry from 22.37 ± 3.78 mm to 17.91 ± 3.84 mm (p < 0.001), of which 80% had no residual proptosis or developed subsequent recurrence. Exophthalmic index calculated by radiologic evaluation also improved from a mean preoperative value of 1.32 ± 0.19 to 1.12 ± 0.13 at the 6-month interval after surgery (p < 0.001). Before surgery, 19 (45%) patients had a relative afferent pupillary reaction with improvement in 9 (24%) after surgery. Of the 14 (33%) patients with preoperative ocular motility deficit, 7 (16%) had resolution of ocular motility deficit postoperatively. The most common surgical complications were temporalis muscle atrophy with temporal hollowing (14%), wound infection (7%), neurogenic strabismus secondary to trochlear nerve palsy (5%), restrictive strabismus (5%), and aponeurotic blepharoptosis (5%). Multidisciplinary frontotemporal orbitozygomatic for resection of SOM is a safe and effective means of tumor removal. It can provide improved visual acuity and proptosis metrics, as well as relief of optic neuropathy and ocular motility deficits.

ObjectiveTo evaluate the association between hormone therapy and the development, progression, and treatment response of spheno-orbital meningiomas (SOMs) and to identify current evidence gaps in clinical management.MethodsA systematic literature review was conducted using an advanced search of PubMed, Embase, Scopus, and Cochrane databases for articles published in English. The selection process adhered to the PRISMA guidelines. Inclusion criteria targeted original research published from inception to May 2025, which discussed hormone therapy exposure and SOMs. Study quality was assessed with Joanna Briggs Institute critical appraisal tools appropriate to each study design.ResultsTwenty articles were retrieved. After screening, 10 studies met the inclusion criteria and were analyzed. The studies comprised 2 case reports, 1 prospective cohort, and 7 retrospective cohorts, totaling 315 patients with SOMs. The gender distribution was predominantly female (166 women, 4 men) in the 7 studies that reported mean age data for patients with SOM. The patients had a mean (SD) history of 12.6 (3.67) years of hormone therapy exposure, with 90% of the therapies being progestins. Six studies reported a decrease in the SOM volume after hormone therapy cessation, and 4 studies documented a decrease in the soft tissue component with progression or stabilization of the intraosseous component. The therapeutic goal was surgical resection. Subtotal resection was associated with higher recurrence than total resection, especially when the residual tumor included soft tissue. Conservative management, involving hormone therapy cessation, was reported in 3 cases. Data were limited regarding progesterone receptor status and the use of radiation therapy.ConclusionsCurrent evidence suggests that hormone therapy, particularly long-term exposure to progestins, may contribute to the development and progression of SOMs. Although cessation of hormone therapy can result in partial tumor regression, especially in soft tissue components, surgical resection remains the primary treatment. Hybrid strategies that combine hormone therapy cessation with surgery may be beneficial in selected cases, although prospective data are lacking. Standardized clinical guidelines and further studies are needed to clarify the role of hormone therapy in the management of SOM.

While several approaches have been described for optic nerve decompression, the endoscopic endonasal route is gaining favor because it provides excellent exposure of the optic canal and the orbital apex in a minimally invasive manner. Very few studies have detailed the experience with nontraumatic optic nerve decompressions, whereas traumatic cases have been widely documented. Herein, the authors describe their preliminary experience with endoscopic endonasal decompression for nontraumatic optic neuropathies (NONs) to determine the procedure's efficacy and delineate its potential indications and limits. The medical reports of patients who had undergone endoscopic endonasal optic nerve and orbital apex decompression for NONs at the Lyon University Neurosurgical Hospital in the period from January 2012 to March 2014 were reviewed. For all cases, clinical and imaging data on the underlying pathology and the patient, including demographics, preoperative and 6-month postoperative ophthalmological assessment results, symptom duration, operative details with video debriefing, as well as the immediate and delayed postoperative course, were collected from the medical records. Eleven patients underwent endoscopic endonasal decompression for NON in the multidisciplinary skull base surgery unit of the Lyon University Neurosurgical Hospital during the 27-month study period. The mean patient age was 53.4 years, and there was a clear female predominance (8 females and 3 males). Among the underlying pathologies were 4 sphenoorbital meningiomas (36%), 3 optic nerve meningiomas (27%), and 1 each of trigeminal neuroma (9%), orbital apex meningioma (9%), ossifying fibroma (9%), and inflammatory pseudotumor of the orbit (9%). Fifty-four percent of the patients had improved visual acuity at the 6-month follow-up. Only 1 patient whose sphenoorbital meningioma had been treated at the optic nerve atrophy stage continued to worsen despite surgical decompression. The 2 patients presenting with preoperative papilledema totally recovered. One case of postoperative epistaxis was successfully treated using balloon inflation, and 1 case of air swelling of the orbit spontaneously resolved. Endoscopic endonasal optic nerve decompression is a safe, effective, and minimally invasive technique affording the restoration of visual function in patients with nontraumatic compressive processes of the orbital apex and optic nerve. The timing of decompression remains crucial, and patients should undergo such a procedure early in the disease course before optic atrophy.

Background: Sphenoid sinus is the most inaccessible paranasal sinus, enclosed within the sphenoid bone and intimately related to numerous vital neural and vascular structures. Anatomic variation of the sphenoid sinus is well documented and may complicate surgery in such a place. Objective: To outline the surgically risky anatomic variants of the sphenoid sinus as well as the variable relationships between the sinus and related neurovascular structures, for the safe removal of intrasphenoid and pituitary lesions. Materials and Methods: We undertook a prospective review of 300 paranasal sinus CT scans of Libyan patients; coronal CT scans were obtained by special parameter techniques. We assessed pneumatization of pterygoid process (PP), anterior clinoid process (ACP), and greater wing of sphenoid (GWS); we also examined protrusion and dehiscence of internal carotid artery (ICA), optic nerve (ON), maxillary nerve (MN), and vidian nerve (VN) into the sphenoid sinus cavity. Results: Pneumatization of PP, ACP, and GWS were seen in 87 (29%), 46 (15.3%), and 60 patients (20%), respectively. Protrusion of ICA, ON, MN, and VN were noticed in 123 (41%), 107 (35.6%), 73 (24.3%), and 81 patients (27%), respectively; dehiscence of these structures was encountered in 90 (30%), 92 (30.6%), 39 (13%), and 111 patients (37%), respectively. Statistically, there was a highly significant association between ACP pneumatization and ICA protrusion, ACP pneumatization and ON protrusion, PP pneumatization and VN protrusion; and GWS pneumatization and MN protrusion (p-value < 0.001). Conclusion: The sphenoid sinus is highly variable; this variability necessitates a comprehensive understanding of the regional sphenoid sinus anatomy by a detailed CT scan sinus examination before surgery in and around the sinus. This study indicates the possibility of a racial anatomical variation of the sphenoid sinus in the Libyan population.

BackgroundSphenoid sinus is the most inaccessible paranasal sinus, enclosed within the sphenoid bone and intimately related to numerous vital neural and vascular structures. Anatomic variation of the sphenoid sinus is well documented and may complicate surgery in such a place.ObjectiveTo outline the surgically risky anatomic variants of the sphenoid sinus as well as the variable relationships between the sinus and related neurovascular structures, for the safe removal of intrasphenoid and pituitary lesions.Materials and MethodsWe undertook a prospective review of 300 paranasal sinus CT scans of Libyan patients; coronal CT scans were obtained by special parameter techniques. We assessed pneumatization of pterygoid process (PP), anterior clinoid process (ACP), and greater wing of sphenoid (GWS); we also examined protrusion and dehiscence of internal carotid artery (ICA), optic nerve (ON), maxillary nerve (MN), and vidian nerve (VN) into the sphenoid sinus cavity.ResultsPneumatization of PP, ACP, and GWS were seen in 87 (29%), 46 (15.3%), and 60 patients (20%), respectively. Protrusion of ICA, ON, MN, and VN were noticed in 123 (41%), 107 (35.6%), 73 (24.3%), and 81 patients (27%), respectively; dehiscence of these structures was encountered in 90 (30%), 92 (30.6%), 39 (13%), and 111 patients (37%), respectively. Statistically, there was a highly significant association between ACP pneumatization and ICA protrusion, ACP pneumatization and ON protrusion, PP pneumatization and VN protrusion; and GWS pneumatization and MN protrusion (p-value < 0.001).ConclusionThe sphenoid sinus is highly variable; this variability necessitates a comprehensive understanding of the regional sphenoid sinus anatomy by a detailed CT scan sinus examination before surgery in and around the sinus. This study indicates the possibility of a racial anatomical variation of the sphenoid sinus in the Libyan population.

Cystic meningiomas are rare, accounting for 2-7% of all intracranial meningiomas. Little is known regarding whether these meningiomas behave differently compared to solid meningiomas. We sought to study this relatively uncommon imaging appearance of meningioma and to evaluate its clinical significance. A single-institution retrospective cohort study of surgically-treated meningioma patients between 2000 and 2019 was conducted. Cystic meningioma was defined as a tumor with an intratumoral or peritumoral cyst present on preoperative imaging. Demographics, preoperative imaging, histopathology characteristics, operative data, and surgical outcomes were reviewed. Imaging variables, histopathology and outcomes were reported for cystic meningiomas and compared with non-cystic meningiomas. Univariate/multivariable analyses were conducted. Of 737 total meningiomas treated surgically, 38 (5.2%) were cystic. Gross total resection (GTR) was achieved in 84.2% of cystic meningioma patients. Eighty-two percent of cystic meningiomas were WHO grade I (n = 31), 15.7% were grade II and 2.6% were grade III. Most cystic meningiomas had low Ki-67/MIB-1 proliferation index (n = 24, 63.2%). A total of 18.4% (n = 7) patients with cystic meningioma had recurrence compared to 12.2% (n = 80) of patients with non-cystic meningioma (p = 0.228). No significant difference in median time to recurrence was observed between cystic and non-cystic meningiomas (25.4, Q1:13.9, Q3:46.9months vs. 13.4, Q1:8.6, Q3:35.5months, p = 0.080). A small portion of intracranial meningiomas have cystic characteristics on imaging. Cystic meningiomas are frequently WHO grade I, have low proliferation index, and had similar outcomes compared to non-cystic meningioma. Cysts in meningioma may not be a surrogate to determine aggressive meningioma behavior.

The pattern of growth of spheno-orbital meningiomas accounts for the main presenting symptoms, such as proptosis, eye motility deficit, visual impairment, diplopia. As these are benign tumors, the postoperative patient's quality of life is an important factor to consider during the preoperative planning.A detailed literature review of superior eyelid transorbital endoscopic approach for spheno-orbital meningiomas, including our own case, was made. A Medline search up to March 2022 in PubMed online electronic database was made using the following key phrases: "superior eyelid endoscopic transorbital approach spheno-orbital meningiomas," "superior eyelid endoscopic transorbital approach," "spheno-orbital meningiomas endoscopic approach." The inclusion criteria were surgical series, reviews, and case reports in English language, as well as papers written in other languages, but including the abstract in English. Cadaveric studies, multiportal combined approaches for SOM, were excluded. The literature review has disclosed five studies for a total of 65 patients, whose demographic, clinical, pathological, surgical, complications, and outcome data were analyzed.Functional and esthetic outcome data after superior eyelid transorbital approach are the following: improvement of proptosis (100%), of visual deficits (66.66%) and of ocular paresis (75%), with only 11 complications (4 trigeminal dysesthesia, 2 CSF leak, 2 wound complications, 1 upper eyelid necrosis, 1 hemorrhage of surgical field, 1 keratitis) reported, but at the expense of extent of resection (gross total resection 33.39%).Based on the outcome data, the superior eyelid transorbital endoscopic approach results in a suitable operative technique for selected spheno-orbital meningiomas.

Background: To describe and evaluate the surgery results regarding resectability and clinical outcomes oftranscranial supraorbital approach for tumor removal of spheno-orbital meningiomaCase Illustration: A 58-year-old woman presented with proptosis of the right eye since 9 years prior.At initial examination, visual acuity was 6/15 with significant nasal visual field defect. There wereprominent proptosis and inferior globe displacement of the right eye with no palpable mass. A frontoparietalbone deformity was also observed. CT scan examination revealed hyperostosis of sphenoid,frontal, and temporal bone with extensive intraorbital mass with contrast enhancement suggestive ofmeningioma. Incisional biopsy was the performed and confirmed the histopathological diagnosis ofmeningothelial meningioma (WHO grade 1). Transcranial supraorbital in conjunction with lateralorbitotomy was performed in this patient as the definitive treatment for tumor removal of sphenoorbitalmeningioma. Craniotomy and opening of orbital roof were carried out by neurosurgeon toexpose intraorbital region. Total tumor removal was than completed.Conclusion: Transcranial supraorbital approach is an effective surgery for spheno-orbital meningiomaremoval as it offers excellent exposure. A radical resection through transcranial approach can be achievedwith low morbidity, providing a significantly improved clinical outcome in long term period. In thiscase, the spheno-orbital meningioma was grossly resected totally with excellent visual outcome andacceptable cosmetic appearance.Keywords: spheno-orbital meningioma, transcranial approach, tumor resection