Cavernous angioma of the cavernous sinus: imaging findings

Angioma cavernoso do seio cavernoso: achados aos exames de imagem

Cavernous angioma of the cavernous sinus: imaging findings

Angioma cavernoso do seio cavernoso: achados aos exames de imagem

Jose Luiz Furtado de Mendonça^{I, II}; Sergio Lopes Viana^II; Makoto Matsumine^II; Renato Faria e Silva^III; Maria Angelica de Carvalho Barbosa Viana^IV; Flavia Mendes de Oliveira Freitas^II

Department of Magnetic Resonance Imaging, Clinica Radiologica Vila Rica, Brasilia DF - Brazil

^IHospital Santa Lucia

^IIClinica Radiologica Vila Rica

IIIHospital Geral e Ortopedico

IVFundaçao Hospitalar do Distrito Federal

^{Correspondence}

ABSTRACT

Cavernous angiomas (cavernomas) of the cavernous sinus are uncommon, with only scattered reports in literature. Unlike their parenchymatous counterparts, they are intensely enhancing para-sellar masses, iso/hypointense in T1- and hyperintense in T2-weighted images. Differential diagnosis with para-sellar meningiomas and schwannomas can be difficult. We report three cases of this condition, describing findings of diffusion-weighted imaging in this kind of lesion for the first time in literature.

Key words: cavernous angioma, cavernous sinus, magnetic resonance imaging, computed tomography, angiography.

RESUMO

Angiomas cavernosos (cavernomas) do seio cavernoso são lesões incomuns, com poucos relatos na literatura especializada. Ao contrário dos cavernomas parenquimatosos, são massas para-selares intensamente captantes, isointensas a hipointensas em T1 e hiperintensas em T2 nas imagens de ressonância magnética. O diagnóstico diferencial com meningiomas e schwannomas para-selares pode ser difícil. Relatamos os achados de imagem de três casos desta entidade, descrevendo os achados da ressonância magnética ponderada em difusão neste tipo de lesão pela primeira vez na literatura.

Palavras-chave: angioma cavernoso, seio cavernoso, ressonância magnética, tomografia computadorizada, arteriografia.

Cavernous angioma of the cavernous sinus is an extremely rare condition, and its differential diagnosis with tumors, namely para-sellar meningiomas and schwannomas, is often difficult. They are richly vascularized lesions, made up of a network of dilated, thin-walled vessels, and are responsible for less than one percent of all intracranial masses. The advent of magnetic resonance imaging (MRI) raised new diagnostic perspectives for this condition as we report in the study.

METHOD

This study was approved by the committee of Ethics of Clinica Radiologia Vila Rica, Brasilia DF, Brazil.

Three female patients, with 42-years-old (patient 1), 45-years-old (patient 2) and 37-years-old (patient 3), are, respectively, presented with complaints of right-sided cranial nerves deficits (III nerve [patients 2 and 3], V1 nerve [patient 2] and VI nerve [patients 1 and 2]) and headaches. All of them underwent magnetic resonance imaging and cerebral digital subtraction angiography (DSA). Patients 2 and 3 were also submitted to computed tomography (CT). In all patients, MRI revealed well-delimited right para-sellar lesions, uniformly isointense in T1-weighted images and hyperintense in T2-, T2- and FLAIR-weighted images, brightly enhancing after intra-vascular infusion of gadolinium. Diffusion-weighted sequence, performed in patient 3, revealed an isointense mass.

RESULTS

In all cases, internal carotid artery (ICA) was encircled by the lesions, without any significant stenosis (Figs 1, 2 and 3). CT images showed fairly well-circumscribed para-sellar masses, slightly to moderately hyperdense in non-enhanced scans, intensely and almost uniformly enhancing after infusion of intravascular iodinated contrast media (Figs 4 and 5). On magnetic resonance angiography (MRA) of cerebral vessels with 3D-TOF technique, performed in patients 2 and 3, the right-sided components of the circle of Willis were laterally displaced by the cavernous angiomas, without any impairment of blood flow (Fig 2). DSA was normal in patient 1; patients 2 and 3 had a supra-sellar and para-sellar blush in late venous phase, once again without impairment of internal carotid flow (Figs 6 and 7). Aneurysm was excluded in all patients. Operative findings were of well-demarcated, hyperemic, reddish to brownish extra-dural para-sellar masses in all cases. These lesions were collapsible, readily refilling after release of compression. Puncture yielded freely flowing blood. Planes of cleavage with surrounding neurovascular structures were not regarded as safe, and none was resected, given the anticipated surgical bleeding.

DISCUSSION

Cavernous angiomas are also known as cavernomas and cavernous hemangiomas. They are common lesions of the cerebral hemispheres, although they can occur anywhere in the central nervous system. Extra-axial cavernous angiomas are uncommon, and the cavernous sinus is one location in this group. It is most common in women in their fifth decade of life and in Japanese people, being rarely multiple and hereditary (specially in Hispanic-American subjects)^1-8. It is, in fact, a vascular malformation, which can behave like a real tumor when it grows up to the point to compress neighbor structures. Exacerbation of symptoms in pregnant women have been reported, improving after delivery^2,3,9. Presentation complaints most often consist of visual disturbances (retro-orbital pain and headache accompanied of reduced ocular motricity, ptosis, diplopia, exophtalmos and impaired visual acuity), mainly due to compressive effect or enclosure of neurovascular structures, namely the cranial nerves passing through the cavernous sinus^1-4,6,10. Seizures, facial numbness and neuralgia may also occur^1,3,5. Clinical evidences of hemorrhage occur in about one third of patients, less often than with intraparenchymal lesions^2,4,7.

Macroscopically, they appear as winy, well-circumscribed, multiloculated masses, richly vascularized, surrounded by a pseudocapsule. Meningiomas and schwannomas, the most important hypotheses in differential diagnosis are, unlike observed in our cases, compactly solid masses, without vascular appearance, and do not yield abundant blood when punctured. Microscopically, they are a honeycomb of vascular spaces which lack muscular and elastic components, lined by a single endothelial layer, without intervening neuronal tissue^5,6.

This entity appears as an iso/hyperdense mass on non-enhanced CT scans, enhancing intensely after infusion of iodinated media in most cases³. Calcification is an occasional finding, and it is most common in meningiomas^5,6,8. Erosion of the sphenoid bone can also be seen. DSA can be normal and show an avascular mass or a discrete to moderate tumoral blush, with feeding vessels originating from branches" of the external carotid or cavernous internal carotid^2-6,9,10. The internal carotid artery is often encircled by the lesion in its cavernous portion, usually maintaining its normal caliber.

Unlike cerebral cavernous angiomas, their cavernous sinus counterparts do not have a pathognomonic appearance on MRI. Findings usually are of well-delimited para-sellar lesions, hypo or isointense in T1-weighted images and brightly hyperintense in T2-weighted images. A dumbbell-shaped mass can be seen, with a small supra-sellar component and most of lesion within cavernous sinus^1,4. MRI allows superb evaluation of the relationships among the cavernous angioma and the surrounding structures^1,2. Gradient-echo sequences may be useful to reveal hemorrhagic component. The mass was isointense in diffusion-weighted images in our only patient in which it was performed, and, to our knowledge, this paper is the first so far to report findings of this technique in cavernous angioma of the cavernous sinus. Enhancing pattern after gadolinium infusion in most cases is intense and homogeneous. The most important differential diagnosis (and the most common preoperative misdiagnosis) is para-sellar meningioma. Although differentiation between these two conditions could be difficult, a homogeneously enhancing mass enwrapping the internal carotid artery without significant reduction of its caliber should lead diagnosis towards cavernous angioma. Other possible hypothesis in this context is para-sellar schwannoma. Meningiomas usually are isointense with gray matter both in T1- and T2-weighted images, while schwannomas tend to have lower signal than gray matter in T1- and higher signal in T2-weighted images. Enhancement is prominent both in meningiomas and schwannomas, but tends to be slightly heterogeneous^1,3,6.

Although parenchymal cavernomas are easily resected in most cases, surgical excision of cavernous angiomas is often challenging because of abundant operative bleeding, due to the vascular nature of these lesions^3-5,7,8,10. Predominantly vascular masses and mostly organized ones can be found⁹. Despite successful resections carried out, high mortality rates still remain, and conservative treatment should be also considered. The possibility of a cavernous angioma must be kept in mind, because of different surgical approaches possibly utilized. Radiotherapy may be a useful complement as the primary therapy or as a preoperative adjuvant^{2, 4}.

In conclusion, no-demarcated, homogeneously enhancing, avascular/hypovascular masses of the middle fossa, which are iso/hypointense in T1- and hyperintense in T2-weighted images should raise the hypothesis of cavernous angiomas, despite the higher frequency of para-sellar meningioma. Because of the high mortality when attempting to remove these lesions, radiologists should alert surgeons for this possibility.

Received 19 March 2004, received in final form 9 June 2004. Accepted 29 July 2004.

• 1. Sohn CH, Kim SP, Kim IM, Lee JH, Lee HK. Characteristic MR imaging findings of cavernous hemangiomas in the cavernous sinus. AJNR 2003;24:1148-1151.
• 2. Bristot R, Santoro A, Fantozzi L, Delfini R. Cavernoma of the cavernous sinus: case report. Surg Neurol 1997;48:160-163.
• 3. Momoshima S, Shiga H, Yuasa Y, Higuchi H, Kawase T, Toya S. MR findings in extracerebral cavernous angiomas of the middle cranial fossa: report of two cases and review of the literature. AJNR 1991;12:756-760.
• 4. Suzuki Y, Shibuya M, Baskaya MK, et al. Extracerebral cavernous angiomas of the cavernous sinus in the middle fossa. Surg Neurol 1996;45:123-132.
• 5. Gliemroth J, Missler U, Sephernia A. Cavernous angioma as a rare neuroradiologic finding in the cavernous sinus. J Clin Neurosci 2000;7:542-560.
• 6. Tannouri F, Divano L, Caucheteur V, et al. Cavernous haemangioma in the cavernous sinus: case report and review of the literature. Neuroradiology 2001;43:317-320.
• 7. Meneses MS, Dallolmo VC, Kondageski C, Ramina R, Hunhevicz S, Pedrozo AA. Cirurgia estereotáxica guiada para angiomas cavernosos. Arq Neuropsiquiatr 2000;58:71-75.
• 8. Andrade GC, Prandini MN, Braga FM. Cavernoma gigante: relato de dois casos. Arq Neuropsiquiatr 2002;60:481-486.
• 9. Shi J, Hang C, Pan Y, Liu C, Zhang Z. Cavernous hemangiomas in the cavernous sinus. Neurosurgery 1999;45:1308-1312.
• 10. Goel A, Muzumdar DP, Nitta J. Surgery on lesions involving cavernous sinus. J Clin Neurosci 2001;8(Suppl 1):S71-S77.

Publication Dates

• Publication in this collection
  25 Apr 2006
• Date of issue
  Dec 2004

History

• Accepted
  29 July 2004
• Reviewed
  09 June 2004
• Received
  19 Mar 2004