Intraosseous meningioma of the sphenoid bone

Intraosseous meningioma of the sphenoid bone

Some sphenoid wing meningiomas are associated with a significant hyperostosis of the adjacent sphenoid ridge that may even exceed the size of the intradural mass. The decision-making process and surgical planning based on neuroanatomic knowledge are the mainstays of management of this group of lesions. Given their natural history and biologic behavior, many hyperostosing meningiomas at this location require long-term management analogous to a chronic disease. This is particularly true when making initial decisions regarding treatment and planning surgical intervention, when it is important to take into consideration the possibility of further future interventions during the patient’s life span 1).


The relationship of the development of intraosseous meningioma to the entrapment of dura containing arachnoid cells is discussed in considering the cause of such lesions, and it is stressed that calvarial fractures and cranial sutures may contribute to the entrapment of arachnoidal tissue and later the formation of a meningioma 2).


Intraosseous growth is a unique feature of sphenoorbital meningioma. Quantitative assessment of the biological behavior of intraosseous remnants revealed a continuous slow growth rate independent of the soft tumor component of more than half of SOM. According to our data, application of a multimodal image guidance provided high accuracy and significantly increased the resection rate of the intraosseous component of SOM 3)

A 24-year-old woman presented with subdural hemorrhage, and subsequent radiology depicted an osteolytic mass-like lesion in the sphenoid bone. Intraoperatively, a solid and cystic hemorrhagic lesion mimicking an aneurysmal bone cyst was observed in the sphenoid bone with dural tearing. Frozen cytology showed singly scattered or epithelioid clusters of round to elongated cells intermixed with many neutrophils. Tumor cells had bland-looking round nuclei with rare prominent nucleoli and nuclear inclusions and eosinophilic granular to globoid cytoplasm in capillary-rich fragments. Histology revealed intraosseous meningothelial and microcystic meningioma (World Health Organization grade 1) in right lesser wing of the sphenoid bone. Considering its unusual location and cytologic findings, differential diagnoses included chordomachondromachondrosarcoma, and aneurysmal bone cyst. The present case posed a diagnostic challenge due to possible confusion with these entities 4)


A 43-year-old female presented with a 1 year history of headache, peri-orbital pain, proptosis, and severe vision loss. She had previously undergone subtotal resection of a large Simpson Grade 1 spheno-orbital meningioma 3 years prior at an outside institution. Workup at our institution revealed hyperostosis of the left greater wing of the sphenoid bone and narrowing of the optic canal along with bony enhancement concerning for residual tumor. The patient was given the recommendation from outside institutions for radiation, presumably due to the chronicity of her visual loss. Our institution recommended resection of the residual osseous tumor with orbital reconstruction. Less than 2 weeks after surgery, the patient noted significant improvement in orbital pain and vision. At 3 months, she had regained full and symmetric orbital appearance with no orbital pain. Her visual acuity improved to 20/30 with full visual fields. Conclusion Surgical decompression of the optic canal and orbital contents for tumor related sphenoid wing hyperostosis should be strongly considered, despite an extended duration of visual change and loss. This case report shows that vision can be significantly restored even after symptoms have been present for greater than 6 months 5).


A 30-year-old female patient presented to the Emergency Department (ED) with a six-week history of right eye pain, diplopia on lateral gaze, and proptosis. She had reported progressive onset of symptoms over the past 12 months. Her only previous medical issue was asthma. Haematological and biochemical results were all normal.

Non-contrast CT orbits were undertaken to evaluate for intraconal or extraconal masses or collection. Findings demonstrated poorly marginated diffuse right greater sphenoid wing cortical thickening, resulting in mass effect on the lateral rectus muscle. Post-contrast CT orbits did not show lesional or soft-tissue enhancement. A CT thorax/abdomen/pelvis was undertaken to exclude a primary malignancy.

MRI orbits pre-and post-contrast demonstrated low-signal thickening of the right greater sphenoid wing with lesional and adjacent dural enhancement on post-contrast sequences. 6).


Use of an acrylic jig to aid orbital reconstruction after resection of a sphenoid intraosseous meningioma: a technical note 7)


A 50-year-old female presented to the Neurosurgery clinic with dimness of vision and proptosis of her right eye. Maxillofacial CT showed a hyperostotic mass involving the right sphenoid ridgeanterior clinoid processorbital roof, and lateral wall with mass effect on the intraorbital contents and lateral wall of the sphenoid sinus. MRI of the brain and orbit showed a heterogeneous enhancement of underlying dura and right orbital apex extending into the cavernous sinus. The patient underwent a staged resection in which pathological analysis showed an intraosseous meningioma. When a hyperostotic mass of the skull is encountered, meningioma should be considered in the differential diagnosis. Although primary intraosseous meningiomas are rare benign tumors, they can be associated with morbidity secondary to mass effect. 8)


A 40-year-old man treated for systemic hypertension complained of decreased vision and floaters in his right eye. Initial examination revealed decreased visual acuity to 20/50 of the right eye with a slight dyschromatopsia, but a lack of afferent pupillary defect and normal visual fields. Fundus examination showed the presence of a slightly swollen right optic disc and chorioretinal folds. A diagnosis of presumed anterior ischemic optic neuropathy was made. Symptoms persisted and, five months later, right proptosis was noted. Magnetic resonance imaging revealed a diffuse thickening of the parieto-temporal bone and the greater wing of the sphenoid bone on the right side. Radiological differential diagnosis included fibrous dysplasia and metastasis.

Bone biopsy revealed a grade I intraosseous meningioma. Conservative management was chosen because the lesion was too extensive to be resected and radiotherapy is usually not efficient on grade I meningiomas.

Intraosseous meningiomas are benign tumors which are due to meningeal cells entrapment during vaginal delivery. It is a rare tumor of slow progression. Therapy usually consists of resection and cranioplasty and/or radiotherapy. In the present case, decompression of the optic canal remains feasible in case of further visual loss 9).


A 71-year-old woman with a long history of slowly progressive proptosis was found to have an intraosseous meningioma of the right sphenoid bone. Radiologically, the lesion resembled fibrous dysplasia. The key to the diagnosis is irregularity of the inner table of the skull. The histologic appearance is characteristic. Intraosseous meningioma is one part of the spectrum of diseases known as primary extraneuraxial meningioma. In this paper we discuss the theories of cellular origin as well as the radiologic differential diagnosis 10)


1)

Kirollos RW. Hyperostosing sphenoid wing meningiomas. Handb Clin Neurol. 2020;170:45-63. doi: 10.1016/B978-0-12-822198-3.00027-6. PMID: 32586508.
2)

Van Tassel P, Lee YY, Ayala A, Carrasco CH, Klima T. Case report 680. Intraosseous meningioma of the sphenoid bone. Skeletal Radiol. 1991;20(5):383-6. doi: 10.1007/BF01267669. PMID: 1896882.
3)

Maschke S, Martínez-Moreno M, Micko A, Millesi M, Minchev G, Mallouhi A, Knosp E, Wolfsberger S. Challenging the osseous component of sphenoorbital meningiomas. Acta Neurochir (Wien). 2019 Nov;161(11):2241-2251. doi: 10.1007/s00701-019-04015-y. Epub 2019 Aug 1. PMID: 31368053; PMCID: PMC6820812.
4)

Kim NR, Yie GT. Intraoperative frozen cytology of intraosseous cystic meningioma in the sphenoid bone. J Pathol Transl Med. 2020 Nov;54(6):508-512. doi: 10.4132/jptm.2020.05.21. Epub 2020 Jul 1. PMID: 32601263; PMCID: PMC7674761.
5)

Parish JM, Shields M, Jones M, Wait SD, Deshmukh VR. Proptosis, Orbital Pain, and Long-Standing Monocular Vision Loss Resolved by Surgical Resection of Intraosseous Spheno-Orbital Meningioma: A Case Report and Literature Review. J Neurol Surg Rep. 2020 Jan;81(1):e28-e32. doi: 10.1055/s-0040-1708845. Epub 2020 Mar 31. PMID: 32257766; PMCID: PMC7108951.
7)

Williams JV, Parmar JD, Carter LM, Woodhead P, Corns R. Use of an acrylic jig to aid orbital reconstruction after resection of a sphenoid intraosseous meningioma: a technical note. Br J Oral Maxillofac Surg. 2019 Dec;57(10):1156-1157. doi: 10.1016/j.bjoms.2019.08.026. Epub 2019 Oct 6. PMID: 31594717.
8)

Hussaini SM, Dziurzynski K, Fratkin JD, Jordan JR, Hussain SA, Khan M. Intraosseous meningioma of the sphenoid bone. Radiol Case Rep. 2015 Nov 6;5(1):357. doi: 10.2484/rcr.v5i1.357. PMID: 27307848; PMCID: PMC4898218.
9)

Henchoz L, Borruat FX. Intraosseous meningioma: a rare cause of chronic optic neuropathy and exophthalmos. Klin Monbl Augenheilkd. 2004 May;221(5):414-7. doi: 10.1055/s-2004-812812. PMID: 15162295.
10)

Daffner RH, Yakulis R, Maroon JC. Intraosseous meningioma. Skeletal Radiol. 1998 Feb;27(2):108-11. doi: 10.1007/s002560050347. PMID: 9526778.

Sphenoid sinus mucosal thickening

Sphenoid sinus mucosal thickening

In pituitary apoplexy etiology, there are reports on the appearance of sphenoid sinus mucosal thickening (SSMT) 1) 2)3).

SSMT is otherwise uncommon with an incidence of up to 7% in asymptomatic individuals. The etiology of SSMT in pituitary apoplexy is unclear and may reflect inflammatory and/or infective changes 4).

The mechanism of thickening of the para sellar dura mater and sphenoid sinus mucosa have been considered to be caused by congestion of dural blood flow because of increased cavernous and circular sinus pressure due to a sudden increase in intrasellar pressure.


A study revealed that age, tumor size, and thickened sphenoid sinus mucosa were strongly related to the occurrence of internal carotid artery stenosis in pituitary apoplexy. Among these factors, age had the potential of being an independent predictor of the condition 5).


Two magnetic resonance imaging (MRI) signs of pituitary apoplexy are the “pituitary ring sign” and “sphenoid sinus mucosal thickening”. The occurrence of both these MRI signs together in patients with ischaemic pituitary apoplexy was investigated. A literature review searching the terms “pituitary ring sign” and “sphenoid sinus mucosal thickening” in the context of pituitary apoplexy from 1990 until the present was performed. To be included in the study, each case had to have ischaemic pituitary apoplexy defined as the acute expansion of a pituitary adenoma or, less commonly, in a non-adenomatous gland, from infarction without hemorrhage or very little hemorrhage and a T1-weighted MRI of the brain with contrast that displayed both “sphenoid sinus mucosal thickening” and a “pituitary ring sign” either on an actual study (the author’s cases) or in a figure in an article from the literature that could be reviewed and clearly illustrate these two signs. Twelve cases of ischaemic pituitary apoplexy were found, all with MRI images that showed both of these signs. Ten cases from the literature (3 of which were published by this author) plus an additional 2 recently evaluated in our hospital, totaled the 12 cases. Thus, 5 of the total 12 cases were evaluated by this author. Of these 12 patients, both headache and visual loss were present in 5 patients, headache alone was indicated in 5 patients (10 of the 12 presented with headache), and no initial symptoms identified in 2 patients (incidentally found non-functioning pituitary adenomas on MRI). These findings indicate that each sign (“pituitary ring sign” and “sphenoid sinus mucosal thickening”) may exist alone with or without pituitary apoplexy, yet both signs together in the appropriate clinical context is a strong predictor of pituitary apoplexy 6).


Arita et al. treated two patients with pituitary apoplexy in whom magnetic resonance (MR) images were obtained before and after the episode. Two days after the apoplectic episodes, MR imaging demonstrated marked thickening of the mucosa of the sphenoid sinus that was absent in the previous studies. The relevance of this change in the sphenoid sinus was investigated. Retrospective evaluations were performed using MR images obtained in 14 consecutive patients with classic pituitary apoplexy characterized by acute onset of severe headache. The mucosa of the sphenoid sinus had thickened predominantly in the compartment just beneath the sella turcica, in nine of 11 patients, as ascertained on MR images obtained within 7 days after the onset of apoplectic symptoms. This condition improved spontaneously in all four patients who did not undergo transsphenoidal surgery. The sphenoid sinus mucosa appeared to be normal on MR images obtained from three patients at the chronic stage (> 3 months after onset). The incidence of sphenoid sinus mucosal thickening during the acute stage was significantly higher in the patients with apoplexy than that in the 100 patients without apoplexy. A histological study conducted in four patients who underwent transsphenoidal surgery during the early stage showed that the subepithelial layer of the sphenoid sinus mucous membrane was obviously swollen. The sphenoid sinus mucosa thickens during the acute stage of pituitary apoplexy. This thickening neither indicates infectious sinusitis nor rules out the choice of the transsphenoidal route for surgery 7).


1)

Agrawal B, Dziurzynski K, Salamat MS, Baskaya M. The temporal association of sphenoid sinus mucosal thickening on MR imaging with pituitary apoplexy. Turk Neurosurg. 2012;22(6):785-90. doi: 10.5137/1019-5149.JTN.4273-11.1. PMID: 23208917.
2)

Liu JK, Couldwell WT. Pituitary apoplexy in the magnetic resonance imaging era: clinical significance of sphenoid sinus mucosal thickening. J Neurosurg. 2006 Jun;104(6):892-8. doi: 10.3171/jns.2006.104.6.892. PMID: 16776332.
3) , 7)

Arita K, Kurisu K, Tominaga A, Sugiyama K, Ikawa F, Yoshioka H, Sumida M, Kanou Y, Yajin K, Ogawa R. Thickening of sphenoid sinus mucosa during the acute stage of pituitary apoplexy. J Neurosurg. 2001 Nov;95(5):897-901. doi: 10.3171/jns.2001.95.5.0897. PMID: 11702884.
4)

Waqar M, McCreary R, Kearney T, Karabatsou K, Gnanalingham KK. Sphenoid sinus mucosal thickening in the acute phase of pituitary apoplexy. Pituitary. 2017 Aug;20(4):441-449. doi: 10.1007/s11102-017-0804-z. PMID: 28421421; PMCID: PMC5508043.
5)

Teramoto S, Tahara S, Kondo A, Morita A. Key Factors Related to Internal Carotid Artery Stenosis Associated with Pituitary Apoplexy. World Neurosurg. 2021 Feb 7:S1878-8750(21)00186-8. doi: 10.1016/j.wneu.2021.02.005. Epub ahead of print. PMID: 33567365.
6)

Vaphiades MS. Pituitary Ring Sign Plus Sphenoid Sinus Mucosal Thickening: Neuroimaging Signs of Pituitary Apoplexy. Neuroophthalmology. 2017 Aug 9;41(6):306-309. doi: 10.1080/01658107.2017.1349807. PMID: 29344069; PMCID: PMC5764063.

Sphenoid wing meningioma

It is a type of anterior skull base meningioma.

These intracranial meningiomas may be associated with hyperostosis of the sphenoid ridge and may be very invasive, spreading to the dura of the frontal, temporal, orbital, and sphenoidal regions. Medially, this tumor may expand into the wall of the cavernous sinus, anteriorly into the orbit, and laterally into the temporal bone.

Epidemiology

The relative incidence of meningiomas of the sphenoid ridge is 17%. This tumor usually arises from the lesser wing of the sphenoid bone. Sphenoid wing meningiomas, or ridge meningiomas, are the most common of the basal meningiomas.

Types

Tumors found in the external third of the sphenoid are of two types: en-plaque and globoid meningiomas.
En plaque meningiomas characteristically lead to slowly increasing proptosis with the eye angled downward. Much of this is due to reactive orbital hyperostosis. With invasion of the tumor into the orbit, diplopia is common.
Patients with globoid meningiomas often present only with signs of increased intracranial pressure. This leads to various other symptoms including headache and a swollen optic disc.
see Medial sphenoid wing meningioma or clinoidal meningioma.
see Meningioma en plaque of the sphenoid ridge.
see Sphenoorbital meningioma

Clinical Features

Tumors growing in the inner wing (clinoidal) most often cause direct damage to the optic nerve leading especially to a decrease in visual acuity, progressive loss of color vision, defects in the field of vision (especially cecocentral), and an afferent pupillary defect.
If the tumor continues to grow and push on the optic nerve, all vision will be lost in that eye as the nerve atrophies.
Proptosis, or anterior displacement of the eye, and palpebral swelling may also occur when the tumor impinges on the cavernous sinus by blocking venous return and leading to congestion. Damage to cranial nerves in the cavernous sinus leads to diplopia.
The Ophthalmic nerve (is often the first affected, leading to diplopia with lateral gaze. The patient will have pain and altered sensation over the front and top of the head.
Horner syndrome may occur if nearby sympathetic fibers are involved.
Endocrine testing is important because pituitary insufficiency has been reported to occur in 22% of patients with anterior skull base meningiomas, including thyroid stimulating hormone (TSH), follicle stimulating hormone (FSH), and luteinizing hormone (LH).

Diagnosis

Following the physical exam, the diagnosis is confirmed with neuro-imaging. Either a head CT or MRI with contrast such as gadolinium is useful, as meningiomas often show homogenous enhancement. Angiography looking for signs like stretched arteries may be used to supplement evaluation of vascular involvement and to determine whether embolization would be helpful if surgery is being considered.
On MRI imaging, T1- and T2-weighted sequences have variable signal intensity, but they enhance intensely and homogeneously after injection of gadolinium. They also tend to exhibit hyperostosis and calcifications which can be seen on either CT or MRI imaging. Additionally, the presence of a dural extension (also known as a dural tail) is helpful in distinguishing a meningioma from fibrous dysplasia.

Differential diagnosis

The differential diagnosis for sphenoid wing meningioma includes other types of tumors such as optic nerve sheath meningioma, cranial osteosarcoma, metastases, and also sarcoidosis.

Treatment

Microsurgery may be the most effective method for the large and giant medial sphenoid wing meningiomas 1).
With the improved requirement of postoperative quality of life in patients, intentional incomplete resection should be considered as an acceptable treatment option. Multivariate analysis confirmed that incomplete resection, poor blood supply, lack of adhesion or encasement of adjacent structure were independent predictive factors for favorable postoperative quality of life. An individual treatment strategy could help improved quality of life 2).

Outcome

Large and giant medial sphenoid wing meningiomas that are located deeply in the skull base where they are closely bounded by cavernous sinus, optic nerve, and internal carotid artery make the gross resection hard to achieve. Also, this kind of meningiomas is often accompanied by a series of severe complications.
For medial sphenoid wing meningiomas, visual loss and abnormalities of cranial nerves III, IV, VI, V1, and V2 may occur because the meningioma may have some degree of encasement of these structures as they ride through the cavernous sinus.
Seizures, paresis, and sensory loss may result depending on potential damage to adjacent brain parenchyma for patients with lateral sphenoid wing meningiomas.
Sphenoid wing meningiomas (SWMs) can encase arteries of the circle of Willis, increasing their susceptibility to intraoperative vascular injury and severe ischemic complications.

Case series

2017

A retrospective review of 75 patients surgically treated for SWM from 2009 to 2015 was undertaken to determine the degree of circumferential vascular encasement (0°-360°) as assessed by preoperative magnetic resonance imaging (MRI). A novel grading system describing “maximum” and “total” arterial encasement scores was created. Postoperative MRIs were reviewed for total ischemia volume measured on sequential diffusion-weighted images.
Of the 75 patients, 89.3% had some degree of vascular involvement with a median maximum encasement score of 3.0 (2.0-3.0) in the internal carotid artery (ICA), M1, M2, and A1 segments; 76% of patients had some degree of ischemia with median infarct volume of 3.75 cm 3 (0.81-9.3 cm 3 ). Univariate analysis determined risk factors associated with larger infarction volume, which were encasement of the supraclinoid ICA ( P < .001), M1 segment ( P < .001), A1 segment ( P = .015), and diabetes ( P = .019). As the maximum encasement score increased from 1 to 5 in each of the significant arterial segments, so did mean and median infarction volume ( P < .001). Risk for devastating ischemic injury >62 cm 3 was found when the ICA, M1, and A1 vessels all had ≥360° involvement ( P = .001). Residual tumor was associated with smaller infarct volumes ( P = .022). As infarction volume increased, so did modified Rankin Score at discharge ( P = .025).
Subtotal resection should be considered in SWM with significant vascular encasement of proximal arteries to limit postoperative ischemic complications 3).

2015

The clinical materials of 53 patients with sphenoid wing meningiomas treated microsurgically between January 2008 and January 2012 were analyzed retrospectively. Follow-up period ranged from 6 to 62 months (median, 34 months). Clinical outcomes including postoperative quality of life and recurrence rate were evaluated. Univariate and multivariate statistical analysis were performed among factors that might influence postoperative quality of life.
The mean age of patients was 49 years. Mean tumor size was 3.9cm. Total tumor resection was achieved in 38 cases (71.7%), subtotal in 10 cases (18.9%) and partial resection in 5 cases (9.4%). Within the follow-up period, ten patients (18.9%) had recurrence and three patients (5.7%) died. In univariate analysis, we found the postoperative Karnofshky Performance Score (KPS) improvement was determined by various factors, including extent of tumor resection, peritumoral edema, tumor blood supply, size, adhesion, encasement and preoperative KPS. However, multivariate analysis showed that complete resection, rich blood supply, adhesion to adjacent structure, encasement of neurovascular were independent predictive factors for worse postoperative KPS.
With the improved requirement of postoperative quality of life in patients, intentional incomplete resection should be considered as an acceptable treatment option. Multivariate analysis confirmed that incomplete resection, poor blood supply, lack of adhesion or encasement of adjacent structure were independent predictive factors for favorable postoperative quality of life. An individual treatment strategy could help improved quality of life 4).

Case reports

2014

Endo et al. report the utility of a pulsed water jet device in meningioma surgery. The presented case is that of a 61-year-old woman with left visual disturbance. MRI demonstrated heterogeneously enhanced mass with intratumoral hemorrhage, indicating sphenoid ridge meningioma on her left side. The tumor invaded the cavernous sinus and left optic canal, engulfing the internal carotid artery in the carotid cistern and encased middle cerebral arteries. During the operation, the pulsed water jet device was useful for dissecting the tumor away from the arteries since it was safe in light of preserving parent arteries. The jet did not cause any vascular injury and did not induce vasospasm as shown by postoperative symptomatology and MRIs. With the aid of pulsed water jet, we could achieve total resection of the tumor except for the piece within the cavernous sinus. The patient had no new neurological deficits after the operation 5).
1)

Yang J, Ma SC, Liu YH, Wei L, Zhang CY, Qi JF, Yu CJ. Large and giant medial sphenoid wing meningiomas involving vascular structures: clinical features and management experience in 53 patients. Chin Med J (Engl). 2013 Dec;126(23):4470-6. PubMed PMID: 24286409.
2) , 4)

Ouyang T, Zhang N, Wang L, Li Z, Chen J. Sphenoid wing meningiomas: Surgical strategies and evaluation of prognostic factors influencing clinical outcomes. Clin Neurol Neurosurg. 2015 May 4;134:85-90. doi: 10.1016/j.clineuro.2015.04.016. [Epub ahead of print] PubMed PMID: 25974397.
3)

McCracken DJ, Higginbotham RA, Boulter JH, Liu Y, Wells JA, Halani SH, Saindane AM, Oyesiku NM, Barrow DL, Olson JJ. Degree of Vascular Encasement in Sphenoid Wing Meningiomas Predicts Postoperative Ischemic Complications. Neurosurgery. 2017 Jun 1;80(6):957-966. doi: 10.1093/neuros/nyw134. PubMed PMID: 28327941.
5)

Endo T, Nakagawa A, Fujimura M, Sonoda Y, Shimizu H, Tominaga T. [Usefulness of pulsed water jet in dissecting sphenoid ridge meningioma while preserving arteries]. No Shinkei Geka. 2014 Nov;42(11):1019-25. doi: 10.11477/mf.1436200025. Japanese. PubMed PMID: 25351797.
WhatsApp WhatsApp us
%d bloggers like this: