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|LETTER TO EDITOR
|Year : 2017 | Volume
| Issue : 5 | Page : 1173-1176
Dural-based giant cavernous hemangioma mimicking a meningioma: Lessons learnt
Pavan K Pelluru, Alugolu Rajesh, Megha S Uppin
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India
|Date of Web Publication||6-Sep-2017|
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500 082, Telangana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Pelluru PK, Rajesh A, Uppin MS. Dural-based giant cavernous hemangioma mimicking a meningioma: Lessons learnt. Neurol India 2017;65:1173-6
Cavernous malformations are usually parenchymal lesions developing as an offset of venous anomalies. Dural-based cavernous malformations are a rare clinical entity affecting the central nervous system, usually mimicking a meningioma. The words ‘cavernomas’ and ‘cavernous hemangiomas’ have been used interchangeably in the literature based on histopathological findings. However, they are quite distinct, not just morphologically but also in a host of other arenas [Table 1].
A 26-year old male patient presented with a history of two episodes of partial seizures with secondary generalization. On examination, he had papilledema with no focal neurological deficits. Computed tomography (CT) scan of the brain, plain and contrast, showed a hyperdense mass lesion of size measuring 6 × 4.9 × 4.8 cm involving the left temporoparietal region with surrounding edema, midline shift, and mass effect [Figure 1]a.
|Figure 1: (a) Plain CT scan of the brain showing a hyperdense lesion in the left temporal region with central hypodensity, midline shift, and mass effect. MRI brain: Left temporal fossa reveals a large extra-axial lesion measuring 6 × 4.9 × 4.8 cm, isointense on T1-weighted (b); and hyperintense on T2-weighted image (c); with a cerebrospinal fluid cleft around the lesion, brilliantly enhancing on administration of contrast (d); On diffusion weighted imaging, the lesion is showing no restriction (e); the lesion is supplied by extracranial branches of the middle meningeal artery (f)|
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There were no calcifications or hyperostosis. Magnetic resonance imaging (MRI) of the brain showed a well-defined, dural-based lesion, measuring 6 × 4.9 × 4.8 cm, which was isointense on T1-weighted imaging, and iso- to hyperintense on T2-weighted and fluid attenuated inversion recovery (FLAIR) imaging, located in the left temporoparietal region with surrounding edema, midline shift, mass effect. There was no restriction on diffusion weighted imaging. Gadolinium–contrast T1-weighted imaging showed a brilliantly enhancing lesion with a prominent dural tail [Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e. Magnetic resonance (MR) angiogram showed a prominent dural vascular supply [Figure 1]f. Our provisional diagnosis was a meningioma. The patient underwent a left frontotemporal craniotomy and Simpson grade I excision of the tumor. Intraoperatively, the tumor was soft, fleshy, and highly vascular. The epicentre of the tumor was around the posterior petrous ridge in the middle cranial fossa. Histopathological examination showed proliferating capillary and cavernous type vessels with intraluminal red cells suggestive of a cavernous haemangioma [Figure 2]a,[Figure 2]b,[Figure 2]c. On immunohistochemistry, the tumor cells showed CD 34 positivity indicating an endothelial origin of the tumor [Figure 2]d. The patient has remained asymptomatic with no recurrence of the lesion at the end of 4 years. He did not receive any radiotherapy or chemotherapy in the postoperative period.
|Figure 2: (a-c) Proliferating capillary and cavernous type of vessels with intraluminal red cells. Some of the vessels show hyalinization of the wall. (d) Endothelial cells are positive for CD34|
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Cavernous malformations have been reported to occur in 0.4–0.8% of the general population, accounting for 10–15% of all vascular malformations of the central nervous system., Intracranial cavernous malformations are usually intra-axial in location. Infrequent sites of occurrence reported in the literature are intraventricular, cavernous sinus, optic apparatus, orbit, and skull base dura. Middle cranial fossa is the common site of occurrence, wherein they occur close to the cavernous sinus., A familial incidence is encountered in 10–30% of the cases, and may rarely be inherited as an autosomal dominant trait with variable penetration linked to markers on the long arm of chromosome 7q. Most common location of cavernomas are supratentorial, with the most common presentation being seizures in 60%, progressive neurological deficits in 50%, and hemorrhage in 20% patients [Table 1].
"Popcorn” or “berry” appearance with a rim of signal loss due to hemosiderin is the characteristic appearance of cavernomas on MRI. T1 and T2 weighted signal varies depending on the age of hemorrhage. Gradient echo and susceptibility-weighted imaging are more sensitive and useful in detecting multiple cavernous angiomas (familial variant). Susceptibility-weighted imaging is also highly sensitive in detecting calcification as compared to T1 and T2 images. Pathological characteristics include the presence of sinusoids with a single layer of endothelium and thin collagenous wall with lack of smooth muscle fibres and elastic fibres. Hallmark pathologic appearance is leaky sinusoids with hemosiderin staining and gliotic reaction in the adjacent brain.
Cavernous hemangiomas usually grow toward the middle fossa and sellar regions, encasing neurovascular structures in the same manner as other tumors in this region. Cavernous hemangiomas within the cavernous sinus are divided into two subgroups based on histopathological and operative findings by Shi et al. Subtype A is a soft, very vascular, and pulsating lesion, associated with severe bleeding, histologically having thin-walled vascular channels with scanty connective tissue. Subtype B is a firmer lesion without visible pulsations, histologically having solid parenchyma, well-formed vessels, and connective tissue.
On computed tomographic (CT) imaging, cavernous hemangiomas are noted to be well-circumscribed, slightly hyperdense masses with adjacent edema or significant mass effect. Sclerosing variants of hemangiomas may have calcifications. On MR imaging, cavernous hemangiomas are hypointense on T1-weighted and homogenously hyperintense on T2-weighted imaging, along with brilliant homogenous enhancement on T1-weighted gadolinium contrast studies. In the present case, the lesion was isointense on T1-weighted, iso to hyperintense on T2-weighted with surrounding edema, and brilliantly enhancing on contrast with dural tail sign, which prompted us to make the diagnosis of a meningioma. A hemosiderin ring, as seen in cavernomas, is not seen in cavernous hemangiomas. The closest differential diagnosis is a meningioma [Table 2].
Kocak et al., in 2002, reported a dural-based cavernous haemangioma arising from the superior petrosal sinus. Ito et al., in 2009, reported cerebellar falx as the site of origin. McCormick in 1966 described angiomas of the dura mater. Supratentorial convexity dural-based cavernous angioma is a very rare entity. Perry et al., in 1993, reported a dura-based parietal convexity cavernous malformation mimicking a meningioma with seizures as presentation. Joshi et al., also reported a supratentorial convexity dura-based cavernoma [Table 3].,,,
|Table 3: Reported characteristics and imageological features of cavernous hemangiomas|
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In conclusion, dural-based cavernous hemangiomas are usually sporadic, benign lesions quite distinct from parenchymal cavernomas, closely mimic meningiomas, and form an important component of the differential diagnosis of lesions arising from the dura.
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[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]
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