====== Giant medial sphenoid meningioma ======

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Giant [[medial sphenoid wing meningioma]]s (greater than or equal to 5 cm in maximum dimension) involve the dura of the greater and lesser [[sphenoid wing]]s, the [[anterior clinoid process]] see [[clinoidal meningioma]], the [[sphenoorbital bone]] and the [[middle cranial fossa]]. 

In more extensive cases, they may also involve the [[cavernous sinus]] see [[cavernous sinus meningioma]], the [[orbital apex]] and [[periorbita]], the [[sellar region]]-[[suprasellar region]] and the pterygopalatine temporal infratemporal fossae 
((O’Sullivan MG, van Loveren HR, Tew JM Jr (1997) The surgical
resectibility of meningiomas of the cavernous sinus. Neurosurgery
40:238–244))
((Yasargil MG (1996) Microneurosurgery of CNS tumours. In: Yasargil MG (ed) Microneurosurgery, vol. IVB. George Thieme Verlag, New York, p 136)).

Thus, they are a surgical challenge due to their intricate locations and intimate relation to vital neural structures 
((Al-Mefty O, Ayoubi S, Smith RR (1991) Direct surgery of the
cavernous sinus: patient selection. Acta Neurochir Suppl (Wien)
53:117–121))
((Al-Mefty O, Smith RR (1988) Surgery of tumours invading the
cavernous sinus. Surg Neurol 30:370–381))
((Cusimano M, Sekhar LN, Sen CN, Pomonis S, Wright DC, Biglan A, Jennetta P (1995) The results of surgery for benign tumours of the cavernous sinus. Neurosurgery 37:1–10))
((Dolenc VV (1989) Anatomy of the cavernous sinus. In: Dolenc VV (ed) Anatomy and surgery of the cavernous sinus. Springer, Berlin Heidelberg New York, pp 3–7))
((El-Kalliny M, van Loveren HR, Keller JT, Tew JM Jr (1992) Tumours of the lateral wall of the cavernous sinus. J Neurosurg 77:508–514))
((Inoue T, Rhoton AL Jr, Theele D, Barry ME (1990) Surgical approaches to the cavernous sinus: a microsurgical study. Neurosurgery 26:903–932))
((Kawase T, Toya S, Shiobara R, Kimura C, Nakajima H (1987) Skull base approaches for the meningiomas invading the cavernous sinus. In: Dolenc VV (ed) The cavernous sinus: multidisciplinary approach to vascular and tumorous lesions. Springer-Verlag, Vienna, pp 346–354))
((Krisht A, Barnett DW, Barrow DL, Bonner G (1994) The blood
supply of the intracavernous cranial nerves: an anatomic study.
Neurosurgery 34:275–279))
((Lanzino G, Hirsch WL, Pomonis S, Sen CN, Sekhar LN (1992)
Cavernous sinus tumours: neuroradiologic and neurosurgical
considerations on 150 operated cases. J Neurosurg Sci 36:183–196))
((Perneczky A, Knosp E, Matula C (1988) Cavernous sinus surgery:
approach through the lateral wall. Acta Neurochir (Wien) 92:76–82))
((Umansky F, Valarezo A, Elidan J (1994) The superior wall of the cavernous sinus A microanatomical study. J Neurosurg 81:914–920)).

===== Clinical features =====
The most common clinical presentation of [[clinoidal meningioma]] and [[medial sphenoid wing meningioma]]s are headaches and visual disturbance such as blurred vision, visual field deficit, or optic atrophy (resulting from optic apparatus compression) or diplopia (resulting from oculomotor nerve distortion).
===== Behari scoring system =====
Based on the calculated volume; the degree of associated [[hyperostosis]] and/or more than 50 % [[calcification]] within the tumour mass; the degree of peritumoural oedema; the extent of tumour invasion to the surrounding region; and, the extent of cavernous sinus invasion, a score ranging between 1 and 12 was assigned to every patient
((Behari S, Giri PJ, Shukla D, Jain VK, Banerji D. Surgical strategies for giant
medial sphenoid wing meningiomas: a new scoring system for predicting extent of
resection. Acta Neurochir (Wien). 2008 Sep;150(9):865-77; discussion 877. doi:
10.1007/s00701-008-0006-6. Epub 2008 Aug 27. PubMed PMID: 18754074.
)).

In patients who had a score of ≥7 out of a total of 12, a small residual tumour had to be left due to the significant risk of neurovascular injury if total excision had been attempted. 

Criteria for scoring system: Volume (in cm2 )=(Length×width)2 /2: <400=1; 400 to 800=2; >800=3. 

Based on method by: Kawamoto Y, Uozumi T, Kiya K, Fujioka Y, Kurisu K, Kawamoto K, Sugiyama K (1995) Clinicopathologic growth factors of acoustic neuromas. Surg Neurol 43:546–52
((Kawamoto Y, Uozumi T, Kiya K, Fujioka Y, Kurisu K, Kawamoto K, Sugiyama K (1995) Clinicopathologic growth factors of acoustic neuromas. Surg Neurol 43:546–52)).

Hyperostosis of anterior clinoid or the wing of sphenoid/>50% calcified mass within the tumour: 
Present=1; Absent=0. Oedema: None—0, Up to 2 cm—1, Lobar/hemispheric—2. 

Cavernous sinus extension: Tumour abutting lateral wall of cavernous sinus—0. 

Tumour touching cavernous internal carotid artery—1. 

Tumour encasing cavernous internal carotid artery—2.

Tumour encasing and narrowing cavernous internal carotid. artery—3. 

Tumour encasing and obliterating cavernous internal carotid artery—4. 

Based on classification by: Hirsch WL, Sekhar LN, Lanzino G, Pomonis S, Sen CN (1993) Meningiomas involving the cavernous sinus: Value of imaging for predicting surgical complications. AJR Am J Roentgenol 160:1083–1088
((Hirsch WL, Sekhar LN, Lanzino G, Pomonis S, Sen CN (1993) Meningiomas involving the cavernous sinus: Value of imaging for predicting surgical complications. AJR Am J Roentgenol 160:1083–1088)). 

Extension to extra region (Sellar-suprasellar; Superior orbital fissure-orbit; and, Prepontine-Interpeduncular cistern-Posterior fossa): none–0, single–1, multiple–2, total score 1–12.

==== DeMonte’s grading of tumour resection ====

Grade Definition

I Complete removal of tumour and dural attachment with any
abnormal bone

II Complete removal of tumour and coagulation of its dural
attachment

IIIa Complete removal of intra- and extradural tumour without
resection or coagulation of its dural attachment

IIIb Complete removal of intradural tumour without resection or
coagulation of its dural attachment or any extradural
tumour extensions

IVa Intentional subtotal removal to preserve cranial nerve or
blood vessels with complete removal of its dural attachment

IVb Subtotal removal leaving <10% tumour volume

V Subtotal removal leaving >10% tumour volume or
decompression with or without biopsy

Based on grading by: DeMonte F, Smith H, Al-Mefty O (1994) Outcome of aggressive removal of cavernous sinus meningiomas. J Neurosurg 81:245–251
((DeMonte F, Smith H, Al-Mefty O (1994) Outcome of aggressive removal of cavernous sinus meningiomas. J Neurosurg 81:245–251)).

===== Treatment =====

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The standard frontotemporal approach as well as the extensions of various skull base approaches were utilized for successful removal of giant medial sphenoidal meningiomas greater than or equal to 5 cm. 

When the preoperative radiological score of Behari et al. was more than or equal to 7 out of a maximum of 12, there was considerable difficulty in achieving total excision of the tumour
((Behari S, Giri PJ, Shukla D, Jain VK, Banerji D. Surgical strategies for giant
medial sphenoid wing meningiomas: a new scoring system for predicting extent of
resection. Acta Neurochir (Wien). 2008 Sep;150(9):865-77; discussion 877. doi:
10.1007/s00701-008-0006-6. Epub 2008 Aug 27. PubMed PMID: 18754074.
)).

An extension of the standard frontotemporal craniotomy using an orbitozygomatic osteotomy, as was done in 7 cases in our series, was found to be especially useful for tumours with extensive orbital, suprasellar and prepontine extensions

===== Complications =====
Cranial nerves are often directly invaded or stretched by the tumour so that it may be difficult to detect or preserve them
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Behari et al. had a high incidence of preoperative hemiparesis often related to the large tumour size and extensive [[perifocal edema]]. Four of the patients developed extensive postoperative oedema. One of them in fact required a [[frontal lobectomy]] and bone flap removal in addition to mannitol and other dehydrants. Venous compromise during extradural drilling, coagulation of the pial, parasitized veins to these large tumours and retraction oedema may all have played a part
((Behari S, Giri PJ, Shukla D, Jain VK, Banerji D. Surgical strategies for giant
medial sphenoid wing meningiomas: a new scoring system for predicting extent of
resection. Acta Neurochir (Wien). 2008 Sep;150(9):865-77; discussion 877. doi:
10.1007/s00701-008-0006-6. Epub 2008 Aug 27. PubMed PMID: 18754074.
)).

===== Retrospective case series =====
Mathios et al. performed a retrospective analysis of a consecutive series of [[lateral transorbital approach]] (LTOA)  patients. Seven target zones were identified: 1) the orbit, 2) the lesser sphenoid wing and anterior clinoid, 3) the middle fossa, 4) the lateral wall of the cavernous sinus and Meckel's cave, 5) the infratemporal fossa, 6) the petrous apex, and 7) the anterior fossa. The authors used volumetric analyses of preoperative and postoperative MR and CT imaging data to calculate the volume of bone and tumor removed and to provide detailed ophthalmological, neurological, and cosmetic outcomes.

Of the 20 patients in this [[cohort]], pathology was in zone 2 (n = 10), zone 4 (n = 6), zone 3 (n = 2), zone 1 (n = 1), and zone 5 (n = 1). Pathology was meningioma (n = 10), schwannoma (n = 2), metastasis (n = 2), epidermoid (n = 1), dermoid (n = 1), encephalocele (n = 1), adenoma (n = 1), glioblastoma (n = 1), and inflammatory lesion (n = 1). The goal was gross-total resection (GTR) in 9 patients, all of whom achieved GTR. Subtotal resection (STR) was the goal in 8 patients (5 spheno-orbital meningiomas, 1 giant cavernous sinus/Meckel's cave schwannoma, 1 cavernous sinus prolactinoma, and 1 cavernous sinus dermoid), 7 of whom achieved STR and 1 of whom achieved GTR. The goal was biopsy in 2 patient and repair of encephalocele in 1. Visual acuity was stable or improved in 18 patients and worse in 2. Transient early postoperative diplopia, ptosis, eyelid swelling, and peri-orbital numbness were common. All 9 patients with preoperative diplopia improved at their last follow-up. Seven of 8 patients with preoperative exophthalmos improved after surgery (average correction of 64%). There were no cases of clinically significant (> 2 mm) postoperative enophthalmos. The most frequent postoperative complaint was peri-orbital numbness (40%). There was 1 CSF leak. Most patients were satisfied with their ocular (84%-100% of patients provided positive satisfaction-related responses) and cosmetic (75%-100%) outcomes.

The LTOA is a safe minimal access approach to a variety of paramedian anterior skull base pathologies in several locations. Early follow-up revealed excellent resolution of exophthalmos with little risk of clinically significant enophthalmos. Transient diplopia, ptosis, and peri-orbital numbness were common but improved. Careful case selection is critical to ensure good outcome
((Mathios D, Bobeff EJ, Longo D, Nilchian P, Estin J, Schwartz AC, Austria Q, Anand VK, Godfrey KJ, Schwartz TH. The lateral transorbital approach to the medial sphenoid wing, anterior clinoid, middle fossa, cavernous sinus, and Meckel's cave: target-based classification, approach-related complications, and intermediate-term ocular outcomes. J Neurosurg. 2023 Sep 1;140(3):677-687. doi: 10.3171/2023.6.JNS23678. PMID: 37657097.)).
===== References =====