Journal of Clinical Neuroscience 21 (2014) 968–974
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Clinical Study
Cavernous sinus hemangioma: A fourteen year single institution experience Sumit Bansal, Ashish Suri ⇑, Manmohan Singh, Shashank Sharad Kale, Deepak Agarwal, Manish Singh Sharma, Ashok Kumar Mahapatra, Bhawani Shankar Sharma Department of Neurosurgery, Neurosciences Center, All India Institute of Medical Sciences, New Delhi 110029, India
a r t i c l e
i n f o
Article history: Received 21 February 2013 Accepted 12 September 2013
Keywords: Cavernous sinus Extradural approach Gamma Knife radiosurgery Hemangioma Transcavernous
a b s t r a c t Cavernous sinus hemangioma (CSH) is a rare extra-axial vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors. Their location, propensity for profuse bleeding during surgery, and relationship to complex neurovascular structures are factors which present difficulty in excising these lesions. The authors describe their experience of 22 patients with CSH over 14 years at a tertiary care center. Patients were managed with microsurgical resection using a purely extradural transcavernous approach (13 patients) and with Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) (nine patients). Retrospective data analysis found headache and visual impairment were the most common presenting complaints, followed by facial hypesthesia and diplopia. All but one patient had complete tumor excision in the surgical series. Transient ophthalmoparesis (complete resolution in 6–8 weeks) was the most common surgical complication. In the GKRS group, marked tumor shrinkage (>50% tumor volume reduction) was achieved in two patients, slight shrinkage in five and no change in two patients, with symptom improvement in the majority of patients. To our knowledge, we describe one of the largest series of CSH managed at a single center. Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality and long-term morbidity, GKRS is an additional tool for treating residual symptomatic lesions or in patients with associated comorbidities making surgical resection unsuitable. Ó 2013 Published by Elsevier Ltd.
1. Introduction Cavernous sinus hemangioma (CSH) is a rare extra-axial highly vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors [1]. They are benign, well-encapsulated neoplasms arising within the confines of the cavernous sinus [1–3]. Hemangiomas most often present in middle-aged patients and are more common in women [4]. CSH may present with headache or various neurological features, including visual diminution, diplopia or facial hypesthesia [5]. Although a number of authors have described their extensive experience with the surgical treatment of cavernous sinus lesions with good clinical results [4,6–9], these uncommon lesions remain a challenge for the neurosurgeon, as there are high mortality and morbidity rates associated with uncontrollable and massive hemorrhage during surgery. Current treatment modalities include microsurgical resection, embolization, fractionated radiation therapy and stereotactic radiosurgery (SRS). The optimal management ⇑ Corresponding author. Tel.: +91 11 2659 3538; fax: +91 11 2658 8559. E-mail address: [email protected] (A. Suri). http://dx.doi.org/10.1016/j.jocn.2013.09.008 0967-5868/Ó 2013 Published by Elsevier Ltd.
strategy is still a matter of controversy [10]. Complete resection of CSH is potentially curative but may be complicated by profuse intra-operative bleeding and new cranial neuropathies [1,11]. Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) has been performed for high-risk or residual CSH. The purpose of this study is to report our experience of removal of CSH through a completely extradural transcavernous approach, with use of GKRS for smaller lesions or medically unfit patients.
2. Patients and methods We analyzed 22 patients with CSH treated over a period of 14 years (from 1999 to 2012), 13 of whom were treated using a purely extradural transcavernous surgical approach, while nine were given primary GKRS. Interestingly, the majority of patients were female, with four male patients (three in the surgical and one in the radiosurgery group). The duration of symptoms ranged from 6 months to 2 years. Contrast-enhanced CT scans and MRI were obtained for all patients. All patients had a pre-operative radiological diagnosis of CSH.
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In the surgical series, patients were treated using an extradural transcavernous approach. The precise access corridor to the tumor in the cavernous sinus depended on the exact location of the tumor and the location of the maximum tumor bulge in the lateral wall of the cavernous sinus. Post-operative MRI to determine the extent of tumor removal was obtained for all patients in the surgical series. In the GKRS series, nine patients were treated using the Leksell Gamma Knife. Treatment planning and radiation dosimetry were done with a goal of conformal and selective coverage of the lesion with a 50% prescription isodose line using the multi-isocenter technique. After GKRS, all patients were discharged from hospital within 24 hours. All patients were scheduled for regular clinical and radiological follow-up every 6 months during the first posttreatment year, and once per year thereafter.
patient who underwent partial resection because of profuse blood loss. Post-operative morbidity included transient cranial nerve dysfunction for 2 to 3 months in all but three patients. One patient developed permanent VI nerve palsy. Vision improved in all patients with pre-operative visual diminution, but not in those with pre-operative complete vision loss. Post-operative MRI revealed gross total excision in 12 patients (Fig. 1). Histopathology revealed CSH in all patients. All patients were evaluated clinically and by imaging 3 months after surgery and then on an annual basis. Follow-up MRI was compared with the pre-operative images and tumor dimensions were measured in the axial, sagittal, and coronal planes. The follow-up period ranged from 3–48 months (mean, 21 months).
3. Results
3.2. Radiosurgical technique
Of the 22 patients, 14 presented with headache, eight with visual impairment, two with facial hypesthesia and seven with diplopia. All patients were evaluated pre-operatively with contrast-enhanced CT scan and MRI. Contrast-enhanced CT scans revealed iso- to hyper-dense expansile lesions in the region of the cavernous sinus and middle cranial fossa. There were signs of variable bony erosion of the anterior clinoid process, sphenoid ridge, or apex of the petrous bone. MRI revealed well-demarcated expansile parasellar lesions, which were hypo- or iso-intense on T1-weighted images and hyperintense on T2-weighted images, with marked homogeneous enhancement after intravenous administration of contrast in all patients. The tumors had well-defined borders and extended variably into the sella and superior orbital fissure.
In the nine GKRS patients the Gamma Knife model B was used up to the year 2010 (n = 6) and the Perfexion model was used thereafter (n = 3) (Table 2). Three patients were medically unfit for surgery so GKRS was chosen, while the other six patients were included because of patient preference and small lesion size. Visualization of the lesion within the cavernous sinus at the time of radiosurgery was attained with MRI using axial 1.0 mm slices of modified time-of-flight and field echo images with and without gadolinium enhancement, as well as coronal 2.0 mm slices of T2weighted images. Treatment planning and radiation dosimetry were done with a goal of conformal and selective coverage of the lesion with a 50% prescription isodose line using the multi-isocenter technique. The mean marginal dose constituted 16.8 Gy (range 12–22.3 Gy). The irradiation dose for the optic pathways was kept below 8 Gy. The median number of iso-centers used to formulate a conformal radiosurgery dose plan was seven (range 3–13). The median target volume was 6.3 cc (range, 2.3–13.3 cc). After GKRS, all patients were discharged from hospital within 24 hours. All patients were scheduled for regular clinical and radiological followup every 6 months during the first post-treatment year, and once per year thereafter. The mean follow-up period ranged from 6– 35 months (mean 17 months). The most recent MRI demonstrated marked tumor shrinkage (>50% tumor volume reduction) in two patients, slight shrinkage in five and no change in two patients (Fig. 2). No tumors showed transient enlargement after GKRS. In the two tumors in which significant shrinkage was attained, a marked tumor volume decrease had occurred by 10 months in one patient and after 35 months in another patient. At the time of writing no patient had experienced tumor recurrence. Headache improved in five patients. Among the five patients with pre-operative ocular movement disturbances, complete remission of symptoms was obtained in three and no change was observed in two patients. Five patients had visual diminution in their pre-radiosurgery evaluation, and two of these patients experienced improvement in their vision.
3.1. Surgical technique All 13 surgical patients were approached through a fronto-temporal osteoplastic craniotomy with or without orbito-zygomatic osteotomy (Table 1). The sphenoid ridge was removed using a high-speed pneumatic drill (Midas Rex; Medtronic, Minneapolis, MN, USA) and the superior orbital fissure was opened. The tentorial duplicature over the base of the anterior clinoid process was peeled from the bone. The anterior clinoid process was drilled extradurally, the optic canal was deroofed, and the extra-cavernous extradural internal carotid artery (ICA) was exposed. The temporal base was drilled, the foramen spinosum was opened, and the middle meningeal artery was coagulated and cut. The meningeal layer of the lateral wall of the cavernous sinus was peeled away from the inner membranous layer using sharp and blunt dissection. The cranial nerves in the lateral wall of the cavernous sinus were exposed (cranial nerves III and IV, as well as V1, V2, and V3). The tumor was bulging either through the lateral triangle (Parkinson’s triangle, between cranial nerves IV and VI or the anterolateral triangle (Mullan’s triangle, between the V1 and V2 nerves). The vascular tumor was removed by rapid decompression using an ultrasonic surgical aspirator and dissected along the cranial nerves. The usual feeders from the meningo-hypophyseal trunk of the intra-cavernous ICA were exposed and coagulated early in the course of the surgery. Cranial nerve VI was seen in close proximity to the ICA. The medial extension of the lesion into the sella was removed. Hemostasis was achieved using gentle packing with gelatin (Spongstan; Johnson & Johnson Medical, New Yorkshire, UK), degraded cellulose (Surgicel; Johnson & Johnson Medical) and hemostatic matrix (Floseal; Baxter Biosurgery, IL, USA), and blood loss was replaced using a cell saver (Autotranfusion unit; Didecon, Mirandola, Italy). Gross total excision was achieved in all but one
4. Discussion 4.1. Patient population and clinical features CSH are usually diagnosed in middle-aged patients [12–13]. The majority of reported cases occur in women [4,12]. Similarly, the majority (n = 18) of our patients were women, with four men (three in the surgical and one in the radiosurgery group). The mean age of our patient group was 42 years, with a range from 26 to 58 years. Headache, cranial neuropathies (especially cranial nerves III, IV, V2, V3, and VI), poor vision, diplopia, ptosis, retro-orbital pain, exophthalmos, endocrinopathy, trigeminal neuralgia, and
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Table 1 Demographics and characteristics of cavernous sinus hemangioma patients in the surgical group Patient
Age (yr)/sex
Side
Symptoms
Clinical signs
Extent of resection
Complications
Follow-up status
Followup (months)
1
41/F
Left
19
Left
EOM full
12
3
50/F
Right
Transient 3rd and 4th nerve palsy Transient complete ophthalmoplegia None
EOM full
42/F
EOM full
48
4
36/F
Right
V/a, R: 6/9; L FC: 3ft; L facial 10% sensory loss V/a, R: 6/18; L PL + L 3rd nerve palsy V/a, L: 6/36; R 6th nerve palsy V/a: B/l 6/9; R 6th nerve palsy
GTR
2
Headache: 1 yr; poor vision in L eye: 6 mo; L facial hypesthesia: 6 mo Headache: 1 yr; L ptosis: 8 mo; poor vision, L eye: 2 mo Headache: 6 mo; bilateral poor vision: 6 mo R PL + Diplopia: 6 mo
STR
Permanent 6th nerve palsy
30
5
38/F
Left
Headache: 1 yr
No deficits
GTR
14
6
38/F
Right
EOM full
24
46/F
Left
V/a, R: 6/9; V/a, L: 6/60; R V1 and V2: 25% sensory loss R 3rd and 6th nerve palsy
GTR
7
Headache: 2 yr; poor vision R eye and diplopia: 6 mo; R facial hypoesthesia: 6 mo Diplopia: 6 mo
Transient complete ophthalmoplegia Transient 3rd nerve palsy
Permanent 6th nerve palsy EOM full
EOM full
19
8
58/F
Left
Poor vision L eye: 14 mo
V/a, L: HMCF
GTR
28
9
26/F
Left
L proptosis: 5 yr; Headache: 3 yr
GTR
None
10
45/M
Left
Headache: 2 yr; Diplopia: 1 yr
GTR
11
36/F
Right
Headache: 3 mo
V/a, R: 6/9; V/a, L: 6/18; EOM: full; L proptosis V/a, B/L: 6/9; L 6th nerve palsy None
EOM full
15
12
32/M
Left
Headache: 3 mo
None
GTR
EOM full
3
13
50/M
Left
Vision loss L eye: 1 yr
Left PL
GTR
Transient complete ophthalmoplegia Transient R 6th nerve palsy Transient 6th nerve palsy, 20% V2 sensory loss Transient complete ophthalmoplegia
EOM full, vision improved Proptosis resolved EOM full
EOM full
3
GTR GTR
GTR
GTR
Transient 6th nerve palsy Transient L 6th nerve palsy
24 15
B/l = bilateral, EOM = external ocular movements, F = female, FC = finger counting, Ft = feet, GTR = gross total resection, HMCF = hand movement close to face, L = left, M = male, mo = months, PL = perception of light, R = right, STR = subtotal resection, V/a = visual acuity, yr = year, + = present, = absent.
hemi- or mono-paresis have been reported in CSH patients [1]. Of the 22 patients, 14 presented with headache, eight presented with visual impairment, two presented with facial hypesthesia and seven presented with diplopia. In spite of the vascular nature of CSH, hemorrhages are extremely unusual during the natural course of the disease [14].
4.2. Neurological imaging Lesion growth is typically accompanied by mass effect and can result in encasement of the adjacent neurovascular structures [5,15]. CSH is the only lesion that is purely intra-cavernous. Thus, even giant lesions remain within the dural confines of the cavernous sinus. CSH tend to become quite large before the patient manifests signs and symptoms [16]. On CT scans, CSH are isodense or minimally hyperdense, with intense homogenous enhancement with contrast administration. They may show pressure erosion of the petrous bone [15]. On MRI, these lesions are well demarcated and homogenously hypointense to white matter on T1-weighted images. Characteristically, they are brightly hyperintense on T2-weighted images, although a few linear septum-like hypointensities may be identified [1,15]. They enhance homogenously and intensely after contrast administration. As the tumors grow in size, they initially displace, and later encase, the ICA. They are often found to extend toward the sella, inter-cavernous sinus, superior orbital fissure, and Meckel’s cave. On angiography, CSH are usually described as ‘‘occult.’’ However, after prolonged selective contrast injection, a mild tumor blush is visible in most patients [1,17–18]. Intra-cavernous meningiomas and schwannomas are the most important lesions to be considered in the differential diagnosis. As compared with CSH, meningioma is less hyperintense on T2-
weighted imaging, but syncytial meningiomas are very hyperintense on T2-weighted imaging and are difficult to distinguish [19–20]. Schwannomas are hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, but are more heterogeneous in appearance [21]. An enlarged foramen ovale favors a diagnosis of trigeminal schwannoma; similar lesions from cranial nerve III, IV, or VI are rare. 4.3. Management considerations Symptomatic CSH can be considered for microsurgical resection, embolization, fractionated radiation therapy or GKRS. 4.3.1. Microsurgery Linskey and Sekhar reported use of a combined intra- and extradural approach to treat three patients with CSH and total excision was achieved with minimal blood loss and with preservation of cranial nerve function in two patients [1]. They hypothesized that the tumor arises within the cavernous sinus and, when small, takes its blood supply from the intra-cavernous carotid artery. Because cranial nerve VI is the only cranial nerve truly within the cavernous sinus, it will most often run through the substance of the tumor; cranial nerves III, IV, and V (V1, V2, and V3) will always be found stretched over the surface of the tumor in the overlying dura. As the tumor grows, it can extend medially into the sella turcica or contralateral cavernous sinus, superiorly into the suprasellar space, or anteriorly into the orbital apex. As the tumor grows laterally, it gradually dissects between the two layers of dura lining the floor of the middle fossa so that it comes underneath cranial nerve V2 and, eventually, cranial nerve V3. As it grows into the middle fossa, it picks up an additional blood supply from the middle meningeal and accessory middle meningeal arteries. Because of the presence
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Fig. 1. Pre-operative T1-weighted contrast-enhanced (a) axial, (b) sagittal and (c) coronal MRI showing a right cavernous sinus hemangioma and post-operative T1-weighted contrast-enhanced (d) axial, (e) sagittal and (f) coronal MRI showing complete excision.
of the pseudo capsule, there will always be a potential plane between the tumor and the overlying dura and cranial nerves. This plane makes it technically easier to preserve cranial nerves III, IV, and V (V1, V2, V3) when removing a hemangioma than when removing a cavernous sinus meningioma that arises from the dura that invests these nerves. Goel et al. described their experience with 13 patients with CSH, seven of whom were operated on using Dolenc’s technique of an entirely extradural approach in 2003 [4]. Of these, 12 patients had a complete tumor resection. The cranial nerve outcome in their series was poor, perhaps because of the large size of the tumors at diagnosis. Zhou et al. reported 20 surgically treated cases of CSH, 13 of which were treated using the extradural approach [22]. Complete
tumor removal was achieved in 12 patients, and at a mean follow-up of 3 years, all patients in this group improved without tumor recurrence. Shi et al. retrospectively analyzed 10 patients with CSH [23]. Among the 10 patients, total tumor removal was performed in four patients, partial removal was performed in two patients, and four patients had tumor biopsies. Both patients who underwent partial removal developed complete opthalmoplegia and diminished sensation in the distribution of cranial nerve VI after surgery; one patient developed contralateral paralysis. A previous series with seven patients by the senior author (A.S.) in 2007 detailed their technique and recommended rapid decompression of the tumor rather than en bloc resection to minimize retraction on the cranial nerves in the lateral wall of the cavernous
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Table 2 Demographics and clinical characteristics of cavernous sinus hemangioma patients in the radiosurgery group Radiation dose (Gy)
Followup period (mo)
Imaging response
Clinical response
15
12
Same
15
12
3.3
15
35
None
2.3
15
35
Decreased size Decreased size Significantly decreased size Decreased size
None
13.3
12
20
5.93
16.9
12
None
5.82
22.3
11
Craniotomy done at other centre but procedure abandoned because of heavy bleeding None
3.89
21.3
10
7.39
19.4
6
Patient
Age (yr)/ sex
Associated comorbidity
Pre-radiosurgery signs and symptoms
Previous procedures
1
46/F
None
None
2
40/F
None
3
50/F
Hypertension
4
27/F
None
5
38/F
None
6
42/F
None
L ptosis: 1 yr; Left 3rd nerve palsy V/a R: HMCF; V/a L: 6/36; L ophthalmoplegia Headache: 2 mo; Poor vision R eye: 2 mo; V/a R: 6/36; V/a L: 6/18 Headache: 2 mo; R ptosis and diplopia: 1 mo; V/a R: 6/ 9; V/a L: 6/6; R 3rd nerve palsy Headache: 1 yr; V/a R: 6/18; V/a L: 6/12 Headache: 1 yr
None
7
50/M
None
Headache: 8 mo
8
46/F
Hypertension; hypothyroidism
Diplopia and R ptosis: 2 yr; R 3rd nerve palsy
9
48/F
Hypertension; R MCA stroke (on aspirin)
Poor vision and restriction of R eye movements; V/a R: 6/ 60; V/a L: 6/18
Tumor volume (cc) 2.9
Craniotomy and partial excision None
13
Decreased size (30%) Same size Decreased size Significantly decreased size Same size
Same Headache improved; vision improved Headache improved; R 3rd nerve palsy improved Headache improved Headache improved Headache improved Diplopia improved
Eye movements improved; vision improved
F = female, HMCF = hand movement close to face, L = left, M = male, MCA = middle cerebral artery, mo = months, R = right, V/a = visual acuity, yr = year.
sinus [24]. The authors also stress the importance of early access to the arterial feeders from the meningo-hypophyseal trunk of the inter-cavernous ICA because early coagulation of the vessels reduces tumor vascularity and blood loss. The other important points include maximum exposure of the cranial base for access to the cavernous sinus, dissection of the meningeal layer of the cavernous sinus lateral wall from the inner membranous layer, and minimizing the handling of the oculomotor nerve. The authors also identified two gross pathological subtypes, which differed in surgical difficulty; which were the soft type with abundant vasculature, and the solid type with abundant interstitial tissue, for which bleeding control was easier. In the present surgical series, gross total excision was achieved in all but one patient who underwent partial resection because of profuse blood loss. The ideal treatment is total tumor removal with the preservation of neurological function. However the surgery is difficult because of the complex structures of the cavernous sinus, encased cranial nerves and ICA, and potential for severe intra-operative bleeding [3,11,17,18,25,26]. As a result, surgical mortality and morbidity rates as high as 36% to 38% have been reported in older series [11,27]. In the present series, post-operative morbidity included transient cranial nerve dysfunction for 2 to 3 months in all but three patients. One patient developed permanent VI nerve palsy. Vision improved in all patients with pre-operative visual diminution but not in those with pre-operative complete vision loss. Rapid decompression of the tumor and early control of tumor vascularity on its medial side are keys to the successful removal of giant tumors [2]. Some surgeons have used hypothermia, vascular occlusion, and intra-operative hypotension during surgery [28]. The surrounding compressed cavernous sinus and a feeder from the meningo-hypophyseal trunk of the intra-cavernous ICA, which is exposed and coagulated early during the course of surgery, supply the CSH. Intra-operative controlled normotensive anesthesia (mean arterial blood pressure 70–80 mmHg) was used. Although these tumors are well demarcated by a fibrous pseudocapsule, the peri-operative mortality rate is high as a result
of poorly controlled bleeding. It has been reported to be as high as 12.5% in recent articles [29] but older series reported up to 36%. 4.3.2. Radiosurgery Radiosurgery is a useful adjunctive treatment for non-operable CSH or for residual neoplasm after initial attempts at microsurgical resection, although it is not considered suitable for lesions near the optic pathways [14,21,22,30]. Several authors reported the efficacy of fractionated radiotherapy for intracranial hemangiomas [31– 35]. Nakamura et al. reported three patients with CSH. The mean tumor volume was 2.3 mL and the mean margin dose was 13.3 Gy (range 12–14 Gy) [13]. Follow-up MRI after 3 months showed a reduction of the tumor volume and improvement in the neurological symptoms. Seo et al. reported a patient with a CSH who underwent GKRS [36]. The tumor volume was 8.5 mL, and the dose delivered to the margin was 15 Gy with a 50% isodose. The dose to the optic apparatus was less than 9 Gy. This investigation reported tumor regression on serial imaging over 2 years. Ivanov et al. reported three patients with CSH treated with low-dose (10–12 Gy) GKRS [37]. All the patients demonstrated tumor volume reduction of more than 60% after 12 months of follow-up. The mechanism proposed is that radiation induces endothelial proliferation, vessel wall hyalinization, and subsequent vessel obliteration in addition to direct cyto-toxicity to the tumor cells [38]. If a tumor shows clear neuroimaging characteristics of CSH and the lesion is small, and without features of either meningioma or schwannoma, GKRS can be performed as the primary treatment procedure. In the present series, all nine patients underwent periodic MRI follow-up after GKRS. The mean follow-up period after GKRS was 17 months, (range 6–35 months). The most recent MRI demonstrated marked tumor shrinkage (>50% tumor volume reduction) in two patients, slight shrinkage in five and no change in two patients. No tumors showed transient enlargement after GKRS. In the two tumors in which significant shrinkage was attained, a marked tumor volume decrease had occurred by 10 months in
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Fig. 2. Pre-radiosurgery T1-weighted contrast-enhanced (a, d) axial, (b) sagittal and (c) coronal MRI showing a left cavernous sinus hemangioma and post-radiosurgery T2weighted (e) axial, (f) sagittal and (g) coronal MRI showing significant decrease in lesion size after primary Gamma Knife (Elekta AB, Stockholm, Sweden) radiosurgery.
one patient and after 35 months in the other patient after GKRS. At the time of writing (mean follow-up = 17 months), no patients had experienced tumor recurrence. Headache improved in five patients. Among the five patients with ocular movement disturbances, complete remission of
symptoms was achieved in three and no change was observed in two patients. Two patients with poor vision also experienced improvement in their vision. On the basis of the radiosurgical results for cavernous sinus meningiomas, the treatment of CSH would appear straightforward
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to most neurosurgeons with experience in radiosurgery. In our study, compared with the results of open surgery for these tumors, radiosurgery appears to have significantly less morbidity but it is too early to conclude that radiosurgery is better than microsurgical resection, in view of our early results. Further long term follow-up is required to validate radiosurgery as a primary treatment in all patients with CSH. 5. Conclusions Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality or long term morbidity in patients, GKRS is an additional tool for treating residual symptomatic lesions or patients with associated comorbidities making surgical resection unsuitable. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Linskey ME, Sekhar LN. Cavernous sinus hemangiomas: a series, a review, and an hypothesis. Neurosurgery 1992;30:101–8. [2] Goel A, Muzumdar D, Sharma P. Extradural approach for cavernous hemangioma of the cavernous sinus: experience with 13 cases. Neurol Med Chir (Tokyo) 2003;43:112–9. [3] Sepehrnia A, Tatagiba M, Brandis A, et al. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990;27:151–5. [4] Goel A. The extradural approach to lesions involving the cavernous sinus. Br J Neurosurg 1997;11:134–8. [5] Hashimoto M, Yokota A, Ohta H, et al. Intratumoral injection of plastic adhesive material for removal of cavernous sinus hemangioma. J Neurosurg 2000;93:1078–81. [6] Al-Mefty O, Smith RR. Surgery of tumors invading the cavernous sinus. Surg Neurol 1988;30:370–81. [7] Dolenc VV. Transcranial epidural approach to pituitary tumors extending beyond the sella. Neurosurgery 1997;41:542–52. [8] Dolenc VV. Extradural approach to intracavernous ICA aneurysms. Acta Neurochir Suppl 1999;72:99–106. [9] Eisenberg MB, Al-Mefty O, DeMonte F, et al. Benign nonmeningeal tumors of the cavernous sinus. Neurosurgery 1999;44:949–55. [10] Gonzalez LF, Lekovic GP, Eschbacher J, et al. Are cavernous sinus hemangiomas and cavernous malformations different entities? Neurosurg Focus 2006;21:E6. [11] Namba S. Extracerebral cavernous hemangioma of the middle cranial fossa. Surg Neurol 1983;19:379–88. [12] Suzuki Y, Shibuya M, Baskaya MK, et al. Extracerebral cavernous angiomas of the cavernous sinus in the middle fossa. Surg Neurol 1996;45:123–32. [13] Nakamura N, Shin M, Tago M, et al. Gamma knife radiosurgery for cavernous hemangiomas in the cavernous sinus: report of three cases. J Neurosurg 2002;97:477–80.
[14] Kida Y, Kobayashi T, Mori Y. Radiosurgery of cavernous hemangiomas in the cavernous sinus. Surg Neurol 2001;56:117–23. [15] Sohn CH, Kim SP, Kim IM, et al. Characteristic MR imaging findings of cavernous hemangiomas in the cavernous sinus. AJNR Am J Neuroradiol 2003;24:1148–51. [16] Zabramski JM, Awasthi D. Extraaxial cavernous malformations. In: Awad IA, Barrow DL, editors. Cavernous malformations. Park Ridge III: AANS; 1993. p. 133–144. [17] Mori K, Handa H, Gi H, et al. Cavernomas in the middle fossa. Surg Neurol 1980;14:21–31. [18] Numaguchi Y, Kishikawa T, Fukui M, et al. Prolonged injection angiography for diagnosing intracranial cavernous hemangiomas. Radiology 1979;131:137–8. [19] Bradac GB, Riva A, Schorner W, et al. Cavernous sinus meningiomas: an MRI study. Neuroradiology 1987;29:578–81. [20] Elster AD, Challa VR, Gilbert TH, et al. Meningiomas: MR and histopathologic features. Radiology 1989;170:857–62. [21] Peker S, Kilic T, Sengoz M, et al. Radiosurgical treatment of cavernous sinus cavernous haemangiomas. Acta Neurochir (Wien) 2004;146:337–41. [22] Zhou LF, Mao Y, Chen L. Diagnosis and surgical treatment of cavernous sinus hemangiomas: an experience of 20 cases. Surg Neurol 2003;60:31–7. [23] Shi J, Hang C, Pan Y, et al. Cavernous hemangiomas in the cavernous sinus. Neurosurgery 1999;45:1308–18. [24] Suri A, Ahmad FU, Mahapatra AK. Extradural transcavernous approach to cavernous sinus hemangiomas. Neurosurgery 2007;60:483–8. [25] Dolenc VV. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990; 27:155 (comment). [26] Laws ER Jr. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990; 27:155 (comment). [27] Kudo T, Ueki S, Kobayashi H, et al. Experience with the ultrasonic surgical aspirator in a cavernous hemangioma of the cavernous sinus. Neurosurgery 1989;24:628–31. [28] Goel A, Nadkarni TD. Cavernous haemangioma in the cavernous sinus. Br J Neurosurg 1995;9:77–80. [29] Ohata K, El-Naggar A, Takami T, et al. Efficacy of induced hypotension in the surgical treatment of the large cavernous sinus cavernoma. J Neurosurg 1999;90:702–8. [30] Aversa do Souto A, Marcondes J, Reis da Silva M, et al. Sclerosing cavernous hemangioma in the cavernous sinus: case report. Skull Base 2003;13:93–9. [31] Grosu AL, Nieder C. Stereotactic fractionated radiotherapy for recurrent capillary hemangioma of the cavernous sinus. Strahlenther Onkol 2006;182: 179–82. [32] Maruishi M, Shima T, Okada Y, et al. Cavernous sinus cavernoma treated with radiation therapy – case report. Neurol Med Chir 1994;34:773–7. [33] Miserocchi G, Vaiani S, Migliore MM, et al. Cavernous hemangioma of the cavernous sinus. Complete disappearance of the neoplasm after subtotal excision and radiation therapy. Case report. J Neurosurg Sci 1997;41:203–7. [34] Rigamonti D, Drayer BP, Johnson PC, et al. The MRI appearance of cavernous malformations (angiomas). J Neurosurg 1987;67:518–24. [35] Shibata S, Mori K. Effect of radiation therapy on extracerebral cavernous hemangioma in the middle fossa. Report of three cases. J Neurosurg 1987;67: 919–22. [36] Seo Y, Fukuoka S, Sasaki T, et al. Cavernous sinus hemangioma treated with gamma knife radiosurgery: usefulness of SPECT for diagnosis – case report. Neurol Med Chir (Tokyo) 2000;40:575–80. [37] Ivanov P, Chernov M, Hayashi M, et al. Low-dose gamma knife radiosurgery for cavernous sinus hemangioma: report of 3 cases and literature review. Minim Invasive Neurosurg 2008;51:140–6. [38] Schneider BF, Eberhard DA, Steiner LE. Histopathology of arteriovenous malformations after gamma knife radiosurgery. J Neurosurg 1997;87:352–7.
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Clinical Study
Cavernous sinus hemangioma: A fourteen year single institution experience Sumit Bansal, Ashish Suri ⇑, Manmohan Singh, Shashank Sharad Kale, Deepak Agarwal, Manish Singh Sharma, Ashok Kumar Mahapatra, Bhawani Shankar Sharma Department of Neurosurgery, Neurosciences Center, All India Institute of Medical Sciences, New Delhi 110029, India
a r t i c l e
i n f o
Article history: Received 21 February 2013 Accepted 12 September 2013
Keywords: Cavernous sinus Extradural approach Gamma Knife radiosurgery Hemangioma Transcavernous
a b s t r a c t Cavernous sinus hemangioma (CSH) is a rare extra-axial vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors. Their location, propensity for profuse bleeding during surgery, and relationship to complex neurovascular structures are factors which present difficulty in excising these lesions. The authors describe their experience of 22 patients with CSH over 14 years at a tertiary care center. Patients were managed with microsurgical resection using a purely extradural transcavernous approach (13 patients) and with Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) (nine patients). Retrospective data analysis found headache and visual impairment were the most common presenting complaints, followed by facial hypesthesia and diplopia. All but one patient had complete tumor excision in the surgical series. Transient ophthalmoparesis (complete resolution in 6–8 weeks) was the most common surgical complication. In the GKRS group, marked tumor shrinkage (>50% tumor volume reduction) was achieved in two patients, slight shrinkage in five and no change in two patients, with symptom improvement in the majority of patients. To our knowledge, we describe one of the largest series of CSH managed at a single center. Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality and long-term morbidity, GKRS is an additional tool for treating residual symptomatic lesions or in patients with associated comorbidities making surgical resection unsuitable. Ó 2013 Published by Elsevier Ltd.
1. Introduction Cavernous sinus hemangioma (CSH) is a rare extra-axial highly vascular neoplasm that accounts for 2% to 3% of all cavernous sinus tumors [1]. They are benign, well-encapsulated neoplasms arising within the confines of the cavernous sinus [1–3]. Hemangiomas most often present in middle-aged patients and are more common in women [4]. CSH may present with headache or various neurological features, including visual diminution, diplopia or facial hypesthesia [5]. Although a number of authors have described their extensive experience with the surgical treatment of cavernous sinus lesions with good clinical results [4,6–9], these uncommon lesions remain a challenge for the neurosurgeon, as there are high mortality and morbidity rates associated with uncontrollable and massive hemorrhage during surgery. Current treatment modalities include microsurgical resection, embolization, fractionated radiation therapy and stereotactic radiosurgery (SRS). The optimal management ⇑ Corresponding author. Tel.: +91 11 2659 3538; fax: +91 11 2658 8559. E-mail address: [email protected] (A. Suri). http://dx.doi.org/10.1016/j.jocn.2013.09.008 0967-5868/Ó 2013 Published by Elsevier Ltd.
strategy is still a matter of controversy [10]. Complete resection of CSH is potentially curative but may be complicated by profuse intra-operative bleeding and new cranial neuropathies [1,11]. Gamma Knife radiosurgery (GKRS; Elekta AB, Stockholm, Sweden) has been performed for high-risk or residual CSH. The purpose of this study is to report our experience of removal of CSH through a completely extradural transcavernous approach, with use of GKRS for smaller lesions or medically unfit patients.
2. Patients and methods We analyzed 22 patients with CSH treated over a period of 14 years (from 1999 to 2012), 13 of whom were treated using a purely extradural transcavernous surgical approach, while nine were given primary GKRS. Interestingly, the majority of patients were female, with four male patients (three in the surgical and one in the radiosurgery group). The duration of symptoms ranged from 6 months to 2 years. Contrast-enhanced CT scans and MRI were obtained for all patients. All patients had a pre-operative radiological diagnosis of CSH.
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In the surgical series, patients were treated using an extradural transcavernous approach. The precise access corridor to the tumor in the cavernous sinus depended on the exact location of the tumor and the location of the maximum tumor bulge in the lateral wall of the cavernous sinus. Post-operative MRI to determine the extent of tumor removal was obtained for all patients in the surgical series. In the GKRS series, nine patients were treated using the Leksell Gamma Knife. Treatment planning and radiation dosimetry were done with a goal of conformal and selective coverage of the lesion with a 50% prescription isodose line using the multi-isocenter technique. After GKRS, all patients were discharged from hospital within 24 hours. All patients were scheduled for regular clinical and radiological follow-up every 6 months during the first posttreatment year, and once per year thereafter.
patient who underwent partial resection because of profuse blood loss. Post-operative morbidity included transient cranial nerve dysfunction for 2 to 3 months in all but three patients. One patient developed permanent VI nerve palsy. Vision improved in all patients with pre-operative visual diminution, but not in those with pre-operative complete vision loss. Post-operative MRI revealed gross total excision in 12 patients (Fig. 1). Histopathology revealed CSH in all patients. All patients were evaluated clinically and by imaging 3 months after surgery and then on an annual basis. Follow-up MRI was compared with the pre-operative images and tumor dimensions were measured in the axial, sagittal, and coronal planes. The follow-up period ranged from 3–48 months (mean, 21 months).
3. Results
3.2. Radiosurgical technique
Of the 22 patients, 14 presented with headache, eight with visual impairment, two with facial hypesthesia and seven with diplopia. All patients were evaluated pre-operatively with contrast-enhanced CT scan and MRI. Contrast-enhanced CT scans revealed iso- to hyper-dense expansile lesions in the region of the cavernous sinus and middle cranial fossa. There were signs of variable bony erosion of the anterior clinoid process, sphenoid ridge, or apex of the petrous bone. MRI revealed well-demarcated expansile parasellar lesions, which were hypo- or iso-intense on T1-weighted images and hyperintense on T2-weighted images, with marked homogeneous enhancement after intravenous administration of contrast in all patients. The tumors had well-defined borders and extended variably into the sella and superior orbital fissure.
In the nine GKRS patients the Gamma Knife model B was used up to the year 2010 (n = 6) and the Perfexion model was used thereafter (n = 3) (Table 2). Three patients were medically unfit for surgery so GKRS was chosen, while the other six patients were included because of patient preference and small lesion size. Visualization of the lesion within the cavernous sinus at the time of radiosurgery was attained with MRI using axial 1.0 mm slices of modified time-of-flight and field echo images with and without gadolinium enhancement, as well as coronal 2.0 mm slices of T2weighted images. Treatment planning and radiation dosimetry were done with a goal of conformal and selective coverage of the lesion with a 50% prescription isodose line using the multi-isocenter technique. The mean marginal dose constituted 16.8 Gy (range 12–22.3 Gy). The irradiation dose for the optic pathways was kept below 8 Gy. The median number of iso-centers used to formulate a conformal radiosurgery dose plan was seven (range 3–13). The median target volume was 6.3 cc (range, 2.3–13.3 cc). After GKRS, all patients were discharged from hospital within 24 hours. All patients were scheduled for regular clinical and radiological followup every 6 months during the first post-treatment year, and once per year thereafter. The mean follow-up period ranged from 6– 35 months (mean 17 months). The most recent MRI demonstrated marked tumor shrinkage (>50% tumor volume reduction) in two patients, slight shrinkage in five and no change in two patients (Fig. 2). No tumors showed transient enlargement after GKRS. In the two tumors in which significant shrinkage was attained, a marked tumor volume decrease had occurred by 10 months in one patient and after 35 months in another patient. At the time of writing no patient had experienced tumor recurrence. Headache improved in five patients. Among the five patients with pre-operative ocular movement disturbances, complete remission of symptoms was obtained in three and no change was observed in two patients. Five patients had visual diminution in their pre-radiosurgery evaluation, and two of these patients experienced improvement in their vision.
3.1. Surgical technique All 13 surgical patients were approached through a fronto-temporal osteoplastic craniotomy with or without orbito-zygomatic osteotomy (Table 1). The sphenoid ridge was removed using a high-speed pneumatic drill (Midas Rex; Medtronic, Minneapolis, MN, USA) and the superior orbital fissure was opened. The tentorial duplicature over the base of the anterior clinoid process was peeled from the bone. The anterior clinoid process was drilled extradurally, the optic canal was deroofed, and the extra-cavernous extradural internal carotid artery (ICA) was exposed. The temporal base was drilled, the foramen spinosum was opened, and the middle meningeal artery was coagulated and cut. The meningeal layer of the lateral wall of the cavernous sinus was peeled away from the inner membranous layer using sharp and blunt dissection. The cranial nerves in the lateral wall of the cavernous sinus were exposed (cranial nerves III and IV, as well as V1, V2, and V3). The tumor was bulging either through the lateral triangle (Parkinson’s triangle, between cranial nerves IV and VI or the anterolateral triangle (Mullan’s triangle, between the V1 and V2 nerves). The vascular tumor was removed by rapid decompression using an ultrasonic surgical aspirator and dissected along the cranial nerves. The usual feeders from the meningo-hypophyseal trunk of the intra-cavernous ICA were exposed and coagulated early in the course of the surgery. Cranial nerve VI was seen in close proximity to the ICA. The medial extension of the lesion into the sella was removed. Hemostasis was achieved using gentle packing with gelatin (Spongstan; Johnson & Johnson Medical, New Yorkshire, UK), degraded cellulose (Surgicel; Johnson & Johnson Medical) and hemostatic matrix (Floseal; Baxter Biosurgery, IL, USA), and blood loss was replaced using a cell saver (Autotranfusion unit; Didecon, Mirandola, Italy). Gross total excision was achieved in all but one
4. Discussion 4.1. Patient population and clinical features CSH are usually diagnosed in middle-aged patients [12–13]. The majority of reported cases occur in women [4,12]. Similarly, the majority (n = 18) of our patients were women, with four men (three in the surgical and one in the radiosurgery group). The mean age of our patient group was 42 years, with a range from 26 to 58 years. Headache, cranial neuropathies (especially cranial nerves III, IV, V2, V3, and VI), poor vision, diplopia, ptosis, retro-orbital pain, exophthalmos, endocrinopathy, trigeminal neuralgia, and
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Table 1 Demographics and characteristics of cavernous sinus hemangioma patients in the surgical group Patient
Age (yr)/sex
Side
Symptoms
Clinical signs
Extent of resection
Complications
Follow-up status
Followup (months)
1
41/F
Left
19
Left
EOM full
12
3
50/F
Right
Transient 3rd and 4th nerve palsy Transient complete ophthalmoplegia None
EOM full
42/F
EOM full
48
4
36/F
Right
V/a, R: 6/9; L FC: 3ft; L facial 10% sensory loss V/a, R: 6/18; L PL + L 3rd nerve palsy V/a, L: 6/36; R 6th nerve palsy V/a: B/l 6/9; R 6th nerve palsy
GTR
2
Headache: 1 yr; poor vision in L eye: 6 mo; L facial hypesthesia: 6 mo Headache: 1 yr; L ptosis: 8 mo; poor vision, L eye: 2 mo Headache: 6 mo; bilateral poor vision: 6 mo R PL + Diplopia: 6 mo
STR
Permanent 6th nerve palsy
30
5
38/F
Left
Headache: 1 yr
No deficits
GTR
14
6
38/F
Right
EOM full
24
46/F
Left
V/a, R: 6/9; V/a, L: 6/60; R V1 and V2: 25% sensory loss R 3rd and 6th nerve palsy
GTR
7
Headache: 2 yr; poor vision R eye and diplopia: 6 mo; R facial hypoesthesia: 6 mo Diplopia: 6 mo
Transient complete ophthalmoplegia Transient 3rd nerve palsy
Permanent 6th nerve palsy EOM full
EOM full
19
8
58/F
Left
Poor vision L eye: 14 mo
V/a, L: HMCF
GTR
28
9
26/F
Left
L proptosis: 5 yr; Headache: 3 yr
GTR
None
10
45/M
Left
Headache: 2 yr; Diplopia: 1 yr
GTR
11
36/F
Right
Headache: 3 mo
V/a, R: 6/9; V/a, L: 6/18; EOM: full; L proptosis V/a, B/L: 6/9; L 6th nerve palsy None
EOM full
15
12
32/M
Left
Headache: 3 mo
None
GTR
EOM full
3
13
50/M
Left
Vision loss L eye: 1 yr
Left PL
GTR
Transient complete ophthalmoplegia Transient R 6th nerve palsy Transient 6th nerve palsy, 20% V2 sensory loss Transient complete ophthalmoplegia
EOM full, vision improved Proptosis resolved EOM full
EOM full
3
GTR GTR
GTR
GTR
Transient 6th nerve palsy Transient L 6th nerve palsy
24 15
B/l = bilateral, EOM = external ocular movements, F = female, FC = finger counting, Ft = feet, GTR = gross total resection, HMCF = hand movement close to face, L = left, M = male, mo = months, PL = perception of light, R = right, STR = subtotal resection, V/a = visual acuity, yr = year, + = present, = absent.
hemi- or mono-paresis have been reported in CSH patients [1]. Of the 22 patients, 14 presented with headache, eight presented with visual impairment, two presented with facial hypesthesia and seven presented with diplopia. In spite of the vascular nature of CSH, hemorrhages are extremely unusual during the natural course of the disease [14].
4.2. Neurological imaging Lesion growth is typically accompanied by mass effect and can result in encasement of the adjacent neurovascular structures [5,15]. CSH is the only lesion that is purely intra-cavernous. Thus, even giant lesions remain within the dural confines of the cavernous sinus. CSH tend to become quite large before the patient manifests signs and symptoms [16]. On CT scans, CSH are isodense or minimally hyperdense, with intense homogenous enhancement with contrast administration. They may show pressure erosion of the petrous bone [15]. On MRI, these lesions are well demarcated and homogenously hypointense to white matter on T1-weighted images. Characteristically, they are brightly hyperintense on T2-weighted images, although a few linear septum-like hypointensities may be identified [1,15]. They enhance homogenously and intensely after contrast administration. As the tumors grow in size, they initially displace, and later encase, the ICA. They are often found to extend toward the sella, inter-cavernous sinus, superior orbital fissure, and Meckel’s cave. On angiography, CSH are usually described as ‘‘occult.’’ However, after prolonged selective contrast injection, a mild tumor blush is visible in most patients [1,17–18]. Intra-cavernous meningiomas and schwannomas are the most important lesions to be considered in the differential diagnosis. As compared with CSH, meningioma is less hyperintense on T2-
weighted imaging, but syncytial meningiomas are very hyperintense on T2-weighted imaging and are difficult to distinguish [19–20]. Schwannomas are hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, but are more heterogeneous in appearance [21]. An enlarged foramen ovale favors a diagnosis of trigeminal schwannoma; similar lesions from cranial nerve III, IV, or VI are rare. 4.3. Management considerations Symptomatic CSH can be considered for microsurgical resection, embolization, fractionated radiation therapy or GKRS. 4.3.1. Microsurgery Linskey and Sekhar reported use of a combined intra- and extradural approach to treat three patients with CSH and total excision was achieved with minimal blood loss and with preservation of cranial nerve function in two patients [1]. They hypothesized that the tumor arises within the cavernous sinus and, when small, takes its blood supply from the intra-cavernous carotid artery. Because cranial nerve VI is the only cranial nerve truly within the cavernous sinus, it will most often run through the substance of the tumor; cranial nerves III, IV, and V (V1, V2, and V3) will always be found stretched over the surface of the tumor in the overlying dura. As the tumor grows, it can extend medially into the sella turcica or contralateral cavernous sinus, superiorly into the suprasellar space, or anteriorly into the orbital apex. As the tumor grows laterally, it gradually dissects between the two layers of dura lining the floor of the middle fossa so that it comes underneath cranial nerve V2 and, eventually, cranial nerve V3. As it grows into the middle fossa, it picks up an additional blood supply from the middle meningeal and accessory middle meningeal arteries. Because of the presence
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Fig. 1. Pre-operative T1-weighted contrast-enhanced (a) axial, (b) sagittal and (c) coronal MRI showing a right cavernous sinus hemangioma and post-operative T1-weighted contrast-enhanced (d) axial, (e) sagittal and (f) coronal MRI showing complete excision.
of the pseudo capsule, there will always be a potential plane between the tumor and the overlying dura and cranial nerves. This plane makes it technically easier to preserve cranial nerves III, IV, and V (V1, V2, V3) when removing a hemangioma than when removing a cavernous sinus meningioma that arises from the dura that invests these nerves. Goel et al. described their experience with 13 patients with CSH, seven of whom were operated on using Dolenc’s technique of an entirely extradural approach in 2003 [4]. Of these, 12 patients had a complete tumor resection. The cranial nerve outcome in their series was poor, perhaps because of the large size of the tumors at diagnosis. Zhou et al. reported 20 surgically treated cases of CSH, 13 of which were treated using the extradural approach [22]. Complete
tumor removal was achieved in 12 patients, and at a mean follow-up of 3 years, all patients in this group improved without tumor recurrence. Shi et al. retrospectively analyzed 10 patients with CSH [23]. Among the 10 patients, total tumor removal was performed in four patients, partial removal was performed in two patients, and four patients had tumor biopsies. Both patients who underwent partial removal developed complete opthalmoplegia and diminished sensation in the distribution of cranial nerve VI after surgery; one patient developed contralateral paralysis. A previous series with seven patients by the senior author (A.S.) in 2007 detailed their technique and recommended rapid decompression of the tumor rather than en bloc resection to minimize retraction on the cranial nerves in the lateral wall of the cavernous
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Table 2 Demographics and clinical characteristics of cavernous sinus hemangioma patients in the radiosurgery group Radiation dose (Gy)
Followup period (mo)
Imaging response
Clinical response
15
12
Same
15
12
3.3
15
35
None
2.3
15
35
Decreased size Decreased size Significantly decreased size Decreased size
None
13.3
12
20
5.93
16.9
12
None
5.82
22.3
11
Craniotomy done at other centre but procedure abandoned because of heavy bleeding None
3.89
21.3
10
7.39
19.4
6
Patient
Age (yr)/ sex
Associated comorbidity
Pre-radiosurgery signs and symptoms
Previous procedures
1
46/F
None
None
2
40/F
None
3
50/F
Hypertension
4
27/F
None
5
38/F
None
6
42/F
None
L ptosis: 1 yr; Left 3rd nerve palsy V/a R: HMCF; V/a L: 6/36; L ophthalmoplegia Headache: 2 mo; Poor vision R eye: 2 mo; V/a R: 6/36; V/a L: 6/18 Headache: 2 mo; R ptosis and diplopia: 1 mo; V/a R: 6/ 9; V/a L: 6/6; R 3rd nerve palsy Headache: 1 yr; V/a R: 6/18; V/a L: 6/12 Headache: 1 yr
None
7
50/M
None
Headache: 8 mo
8
46/F
Hypertension; hypothyroidism
Diplopia and R ptosis: 2 yr; R 3rd nerve palsy
9
48/F
Hypertension; R MCA stroke (on aspirin)
Poor vision and restriction of R eye movements; V/a R: 6/ 60; V/a L: 6/18
Tumor volume (cc) 2.9
Craniotomy and partial excision None
13
Decreased size (30%) Same size Decreased size Significantly decreased size Same size
Same Headache improved; vision improved Headache improved; R 3rd nerve palsy improved Headache improved Headache improved Headache improved Diplopia improved
Eye movements improved; vision improved
F = female, HMCF = hand movement close to face, L = left, M = male, MCA = middle cerebral artery, mo = months, R = right, V/a = visual acuity, yr = year.
sinus [24]. The authors also stress the importance of early access to the arterial feeders from the meningo-hypophyseal trunk of the inter-cavernous ICA because early coagulation of the vessels reduces tumor vascularity and blood loss. The other important points include maximum exposure of the cranial base for access to the cavernous sinus, dissection of the meningeal layer of the cavernous sinus lateral wall from the inner membranous layer, and minimizing the handling of the oculomotor nerve. The authors also identified two gross pathological subtypes, which differed in surgical difficulty; which were the soft type with abundant vasculature, and the solid type with abundant interstitial tissue, for which bleeding control was easier. In the present surgical series, gross total excision was achieved in all but one patient who underwent partial resection because of profuse blood loss. The ideal treatment is total tumor removal with the preservation of neurological function. However the surgery is difficult because of the complex structures of the cavernous sinus, encased cranial nerves and ICA, and potential for severe intra-operative bleeding [3,11,17,18,25,26]. As a result, surgical mortality and morbidity rates as high as 36% to 38% have been reported in older series [11,27]. In the present series, post-operative morbidity included transient cranial nerve dysfunction for 2 to 3 months in all but three patients. One patient developed permanent VI nerve palsy. Vision improved in all patients with pre-operative visual diminution but not in those with pre-operative complete vision loss. Rapid decompression of the tumor and early control of tumor vascularity on its medial side are keys to the successful removal of giant tumors [2]. Some surgeons have used hypothermia, vascular occlusion, and intra-operative hypotension during surgery [28]. The surrounding compressed cavernous sinus and a feeder from the meningo-hypophyseal trunk of the intra-cavernous ICA, which is exposed and coagulated early during the course of surgery, supply the CSH. Intra-operative controlled normotensive anesthesia (mean arterial blood pressure 70–80 mmHg) was used. Although these tumors are well demarcated by a fibrous pseudocapsule, the peri-operative mortality rate is high as a result
of poorly controlled bleeding. It has been reported to be as high as 12.5% in recent articles [29] but older series reported up to 36%. 4.3.2. Radiosurgery Radiosurgery is a useful adjunctive treatment for non-operable CSH or for residual neoplasm after initial attempts at microsurgical resection, although it is not considered suitable for lesions near the optic pathways [14,21,22,30]. Several authors reported the efficacy of fractionated radiotherapy for intracranial hemangiomas [31– 35]. Nakamura et al. reported three patients with CSH. The mean tumor volume was 2.3 mL and the mean margin dose was 13.3 Gy (range 12–14 Gy) [13]. Follow-up MRI after 3 months showed a reduction of the tumor volume and improvement in the neurological symptoms. Seo et al. reported a patient with a CSH who underwent GKRS [36]. The tumor volume was 8.5 mL, and the dose delivered to the margin was 15 Gy with a 50% isodose. The dose to the optic apparatus was less than 9 Gy. This investigation reported tumor regression on serial imaging over 2 years. Ivanov et al. reported three patients with CSH treated with low-dose (10–12 Gy) GKRS [37]. All the patients demonstrated tumor volume reduction of more than 60% after 12 months of follow-up. The mechanism proposed is that radiation induces endothelial proliferation, vessel wall hyalinization, and subsequent vessel obliteration in addition to direct cyto-toxicity to the tumor cells [38]. If a tumor shows clear neuroimaging characteristics of CSH and the lesion is small, and without features of either meningioma or schwannoma, GKRS can be performed as the primary treatment procedure. In the present series, all nine patients underwent periodic MRI follow-up after GKRS. The mean follow-up period after GKRS was 17 months, (range 6–35 months). The most recent MRI demonstrated marked tumor shrinkage (>50% tumor volume reduction) in two patients, slight shrinkage in five and no change in two patients. No tumors showed transient enlargement after GKRS. In the two tumors in which significant shrinkage was attained, a marked tumor volume decrease had occurred by 10 months in
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Fig. 2. Pre-radiosurgery T1-weighted contrast-enhanced (a, d) axial, (b) sagittal and (c) coronal MRI showing a left cavernous sinus hemangioma and post-radiosurgery T2weighted (e) axial, (f) sagittal and (g) coronal MRI showing significant decrease in lesion size after primary Gamma Knife (Elekta AB, Stockholm, Sweden) radiosurgery.
one patient and after 35 months in the other patient after GKRS. At the time of writing (mean follow-up = 17 months), no patients had experienced tumor recurrence. Headache improved in five patients. Among the five patients with ocular movement disturbances, complete remission of
symptoms was achieved in three and no change was observed in two patients. Two patients with poor vision also experienced improvement in their vision. On the basis of the radiosurgical results for cavernous sinus meningiomas, the treatment of CSH would appear straightforward
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to most neurosurgeons with experience in radiosurgery. In our study, compared with the results of open surgery for these tumors, radiosurgery appears to have significantly less morbidity but it is too early to conclude that radiosurgery is better than microsurgical resection, in view of our early results. Further long term follow-up is required to validate radiosurgery as a primary treatment in all patients with CSH. 5. Conclusions Although microsurgical resection using an extradural transcavernous approach is considered the treatment of choice in CSH and allows complete excision with minimal mortality or long term morbidity in patients, GKRS is an additional tool for treating residual symptomatic lesions or patients with associated comorbidities making surgical resection unsuitable. Conflicts of interest/disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [1] Linskey ME, Sekhar LN. Cavernous sinus hemangiomas: a series, a review, and an hypothesis. Neurosurgery 1992;30:101–8. [2] Goel A, Muzumdar D, Sharma P. Extradural approach for cavernous hemangioma of the cavernous sinus: experience with 13 cases. Neurol Med Chir (Tokyo) 2003;43:112–9. [3] Sepehrnia A, Tatagiba M, Brandis A, et al. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990;27:151–5. [4] Goel A. The extradural approach to lesions involving the cavernous sinus. Br J Neurosurg 1997;11:134–8. [5] Hashimoto M, Yokota A, Ohta H, et al. Intratumoral injection of plastic adhesive material for removal of cavernous sinus hemangioma. J Neurosurg 2000;93:1078–81. [6] Al-Mefty O, Smith RR. Surgery of tumors invading the cavernous sinus. Surg Neurol 1988;30:370–81. [7] Dolenc VV. Transcranial epidural approach to pituitary tumors extending beyond the sella. Neurosurgery 1997;41:542–52. [8] Dolenc VV. Extradural approach to intracavernous ICA aneurysms. Acta Neurochir Suppl 1999;72:99–106. [9] Eisenberg MB, Al-Mefty O, DeMonte F, et al. Benign nonmeningeal tumors of the cavernous sinus. Neurosurgery 1999;44:949–55. [10] Gonzalez LF, Lekovic GP, Eschbacher J, et al. Are cavernous sinus hemangiomas and cavernous malformations different entities? Neurosurg Focus 2006;21:E6. [11] Namba S. Extracerebral cavernous hemangioma of the middle cranial fossa. Surg Neurol 1983;19:379–88. [12] Suzuki Y, Shibuya M, Baskaya MK, et al. Extracerebral cavernous angiomas of the cavernous sinus in the middle fossa. Surg Neurol 1996;45:123–32. [13] Nakamura N, Shin M, Tago M, et al. Gamma knife radiosurgery for cavernous hemangiomas in the cavernous sinus: report of three cases. J Neurosurg 2002;97:477–80.
[14] Kida Y, Kobayashi T, Mori Y. Radiosurgery of cavernous hemangiomas in the cavernous sinus. Surg Neurol 2001;56:117–23. [15] Sohn CH, Kim SP, Kim IM, et al. Characteristic MR imaging findings of cavernous hemangiomas in the cavernous sinus. AJNR Am J Neuroradiol 2003;24:1148–51. [16] Zabramski JM, Awasthi D. Extraaxial cavernous malformations. In: Awad IA, Barrow DL, editors. Cavernous malformations. Park Ridge III: AANS; 1993. p. 133–144. [17] Mori K, Handa H, Gi H, et al. Cavernomas in the middle fossa. Surg Neurol 1980;14:21–31. [18] Numaguchi Y, Kishikawa T, Fukui M, et al. Prolonged injection angiography for diagnosing intracranial cavernous hemangiomas. Radiology 1979;131:137–8. [19] Bradac GB, Riva A, Schorner W, et al. Cavernous sinus meningiomas: an MRI study. Neuroradiology 1987;29:578–81. [20] Elster AD, Challa VR, Gilbert TH, et al. Meningiomas: MR and histopathologic features. Radiology 1989;170:857–62. [21] Peker S, Kilic T, Sengoz M, et al. Radiosurgical treatment of cavernous sinus cavernous haemangiomas. Acta Neurochir (Wien) 2004;146:337–41. [22] Zhou LF, Mao Y, Chen L. Diagnosis and surgical treatment of cavernous sinus hemangiomas: an experience of 20 cases. Surg Neurol 2003;60:31–7. [23] Shi J, Hang C, Pan Y, et al. Cavernous hemangiomas in the cavernous sinus. Neurosurgery 1999;45:1308–18. [24] Suri A, Ahmad FU, Mahapatra AK. Extradural transcavernous approach to cavernous sinus hemangiomas. Neurosurgery 2007;60:483–8. [25] Dolenc VV. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990; 27:155 (comment). [26] Laws ER Jr. Cavernous angioma of the cavernous sinus: case report. Neurosurgery 1990; 27:155 (comment). [27] Kudo T, Ueki S, Kobayashi H, et al. Experience with the ultrasonic surgical aspirator in a cavernous hemangioma of the cavernous sinus. Neurosurgery 1989;24:628–31. [28] Goel A, Nadkarni TD. Cavernous haemangioma in the cavernous sinus. Br J Neurosurg 1995;9:77–80. [29] Ohata K, El-Naggar A, Takami T, et al. Efficacy of induced hypotension in the surgical treatment of the large cavernous sinus cavernoma. J Neurosurg 1999;90:702–8. [30] Aversa do Souto A, Marcondes J, Reis da Silva M, et al. Sclerosing cavernous hemangioma in the cavernous sinus: case report. Skull Base 2003;13:93–9. [31] Grosu AL, Nieder C. Stereotactic fractionated radiotherapy for recurrent capillary hemangioma of the cavernous sinus. Strahlenther Onkol 2006;182: 179–82. [32] Maruishi M, Shima T, Okada Y, et al. Cavernous sinus cavernoma treated with radiation therapy – case report. Neurol Med Chir 1994;34:773–7. [33] Miserocchi G, Vaiani S, Migliore MM, et al. Cavernous hemangioma of the cavernous sinus. Complete disappearance of the neoplasm after subtotal excision and radiation therapy. Case report. J Neurosurg Sci 1997;41:203–7. [34] Rigamonti D, Drayer BP, Johnson PC, et al. The MRI appearance of cavernous malformations (angiomas). J Neurosurg 1987;67:518–24. [35] Shibata S, Mori K. Effect of radiation therapy on extracerebral cavernous hemangioma in the middle fossa. Report of three cases. J Neurosurg 1987;67: 919–22. [36] Seo Y, Fukuoka S, Sasaki T, et al. Cavernous sinus hemangioma treated with gamma knife radiosurgery: usefulness of SPECT for diagnosis – case report. Neurol Med Chir (Tokyo) 2000;40:575–80. [37] Ivanov P, Chernov M, Hayashi M, et al. Low-dose gamma knife radiosurgery for cavernous sinus hemangioma: report of 3 cases and literature review. Minim Invasive Neurosurg 2008;51:140–6. [38] Schneider BF, Eberhard DA, Steiner LE. Histopathology of arteriovenous malformations after gamma knife radiosurgery. J Neurosurg 1997;87:352–7.
