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Show Journal of Neuro- Ophthalmology 15( 4): 225- 229, 1995. © 1995 Lippincott- Raven Publishers, Philadelphia Cavernous Sinus Hemangioma Clinical and Neuroimaging Features Andrew G. Lee, M. D., Neil R. Miller, M. D., Paul W. Brazis, M. D., and Mark L. Benson, M. D. A patient presented with an isolated left sixth nerve palsy. Magnetic resonance imaging revealed a sharply marginated 3 cm lesion in the left cavernous sinus, which was isointense to gray matter on Tl- weighted images, hyperintense on T2- weighted images, and enhanced with paramagnetic contrast material. Cerebral angiography showed a homogeneous blush fed by an enlarged meningohypophyseal artery. The neuroimaging findings were thought to be most consistent with the diagnosis of a cavernous sinus meningioma. At the time of surgery, a vascular mass was encountered, and a biopsy was consistent with a cavernous hemangioma. This report describes the clinical and neuroimaging features of cavernous sinus hemangiomas that may help to differentiate them from other cavernous sinus lesions. Key Words: Cavernous hemangioma- Cavernous sinus- Magnetic resonance imaging. Manuscript received May 10, 1994; revised June 30, 1994. From the Neuro- ophthalmology Unit ( A. G. L., N. R. M., P. W. B.), The Wilmer Ophthalmological Institute, and the Division of Neuroradiology ( M. L. B.), The Russell H. Morgan Department of Radiology, The Johns Hopkins Medical Institutions, Baltimore, Maryland, U. S. A. Dr. Brazis is now affiliated with the Mayo Clinic in Jacksonville, Florida. This work was supported in part by a grant from the Fight for Sight Research, Division of the National Society to Prevent Blindness in memory of Herbert Tenzer. Address correspondence and reprint requests to Dr. Neil Miller, Neuro- Ophthalmology Unit, The Wilmer Ophthalmological Institute, Maumenee B- 109, The Johns Hopkins Medical Institutions, 600 N. Wolfe St., Baltimore, MD 21287, U. S. A. Cavernous hemangiomas are vascular hamartomas that occasionally arise in the cavernous sinus ( 1,2) and that represent about 3% of all benign tumors in this region ( 3). This report describes the clinical and neuroimaging features of cavernous sinus hemangiomas that may help to differentiate them from other cavernous sinus lesions. CASE REPORT A 31- year- old woman presented with a 2- month history of binocular horizontal diplopia, worse on gaze to the left. There was no clear precipitant for the onset of her symptoms, and she had no other systemic or neurologic complaints. Past medical history was significant for well- differentiated papillary carcinoma of the thyroid gland with metastasis to one paratracheal lymph node diagnosed 11 years earlier. This was treated with total thyroidectomy and right modified radical neck dissection. She had been followed at regular intervals by her medical doctor and had no evidence of recurrence or metastasis at presentation. General physical examination was normal except for a surgical scar on the neck with excavation of both supraclavicular fossae, right more than left. Visual acuity, color vision, visual fields, and pupillary reactions to light and near stimuli were normal. Corneal and facial sensation were normal. Motility examination showed reduced abduction of the left eye ( 55 degrees in the left eye versus 75 degrees in the right eye). In primary position, the patient had an esophoria of 6 prism diopters. On gaze to the right, there was an esophoria of 8 prism diopters and on gaze to the left, there was an esotropia of 20 prism diopters. The ocular fundi were normal. A diagnosis of an isolated left abducens nerve paresis was made. Magnetic resonance ( MR) imaging of the brain 225 226 A. G. LEE ET AL. revealed a sharply marginated, 3- cm lesion in the left cavernous sinus that was isointense to gray matter on Tl- weighted MR images ( Fig. 1). Following intravenous administration of gadolinium- DTPA, the mass enhanced markedly ( Fig. 2). The mass was hyperintense on proton- density ( Fig. 3) and T2- weighted images, surrounded the intracav-ernous portion of the left internal carotid artery and extended into the sella turcica. A few curvilinear flow voids were seen within the tumor, suggesting small vessels. Cerebral angiography showed a hypervascular mass within the left cavernous sinus with upward displacement of the intracavernous portion of the left internal carotid artery. The vascular supply to the mass originated from meningohypophyseal branches of the left internal carotid artery ( Fig. 4). There was no evidence for vascular supply from the external carotid artery. COURSE A preliminary diagnosis of cavernous sinus meningioma was made, and the patient underwent a FIG. 1. Axial T1- weighted MR image shows a mass within the left cavernous sinus that is isointense to gray matter ( arrows). Several small flow voids are present { arrowheads), which suggests that this is a hypervascular lesion. FIG. 2. Axial T1- weighted MR image after intravenous administration of gadolinium- DTPA reveals homogeneous enhancement ( arrows) of the mass with lateral bulging into the medial aspect of the left temporal lobe. left pterional craniotomy, at which time the neurosurgeon encountered a dark vascular mass that was biopsied. Frozen section of the lesion was consistent with a vascular hamartoma, and therefore the surgeon elected not to attempt removal of the remainder of the mass. Permanent pathologic sections revealed numerous irregular vascular channels lined by endothelium and separated by a fibroconnective tissue stroma consistent with a cavernous angioma ( Fig. 5). The specimen demonstrated markedly positive staining for factor VIII of the endothelial walls of the vascular channels, further supporting the diagnosis of an angioma. Postoperatively, the patient had a persistent left abducens nerve paresis, a left Horner syndrome, and left- sided sensory loss in the cutaneous distribution of the ophthalmic division of the trigeminal nerve. DISCUSSION Cavernous hemangiomas only rarely arise in the cavernous sinus, predominantly occurring in women ( 94%) ( 4) in the fifth decade of life ( 5). Al- / Neuro- Ophthalmol, Vol. 15, No. 4, 1995 CAVERNOUS SINUS HEMANGIOMA 227 though benign, cavernous hemangiomas may cause retrobulbar pain, facial numbness, ophthalmoplegia, or a combination of these findings while they are still fairly small ( 1,5). Alternatively, they may remain asymptomatic until they are very large, at which time they may produce signs and symptoms of a typical cavernous sinus syndrome. If they extend superiorly, hypothalamic manifestations such as weight gain, decreased libido, or increased thirst may occur ( 1,3,4,6). Symptoms may fluctuate, becoming more pronounced during periods of strenuous physical activity, perhaps related to vascular engorgement of the tumor within the cavernous sinus ( 7). Occasionally, hemorrhage within the tumor may cause sudden onset or exacerbation of symptoms and signs. Neuroimaging studies may be helpful in the diagnosis of cavernous sinus hemangioma. Computed tomographic ( CT) scans usually show a well- demarcated, homogeneously enhancing mass that may be indistinguishable from a meningioma ( 2,8). However, the lesion characteristically is not calcified, and the surrounding bone typically demonstrates erosion or remodeling, rather than hy- FIG. 3. Axial proton density MR image demonstrates a well- circumscribed hyperintense mass ( arrows) that narrows the left internal carotid artery flow void ( arrowheads) and elevates the anterior cavernous portion out of the plane of this image. FIG. 4. Anterior- posterior view of the left internal carotid arteriogram shows a vascular blush within the left cavernous sinus mass ( arrowheads), which is fed by an enlarged meningohypophyseal artery ( arrow). perostosis. The presence of calcium within the lesion or associated hyperostosis on CT scanning thus favors meningioma, although the absence of these findings does not necessarily differentiate between the two lesions ( 3). Although MR imaging of intracranial hemangiomas usually reveals characteristic changes- iso-or hypointensity on Tl- weighted images; iso- or hyperintensity on T2- weighted images; a " reticulated" appearing core of mixed signal intensity on T2- weighted images; a prominent hypointense rim on Tl- and T2- weighted images; and nonhomoge-neous enhancement after intravenous administration of paramagnetic contrast material- these features are uncommonly seen with hemangiomas in the cavernous sinus ( 2). Instead, such lesions usually show a homogeneous signal intensity on un-enhanced Tl- and T2- weighted images and marked enhancement after the intravenous administration of paramagnetic contrast material. This radiographic pattern was seen in our patient and was thought to be most consistent with a meningioma. Like CT scanning and MR imaging, cerebral angiography may or may not be helpful in distinguishing cavernous hemangiomas from other lesions in the cavernous sinus. One- third of cavernous sinus hemangiomas are angiographically / Neuro- Ophthalmol, Vol. 15, No. 4, 1995 228 A. G. LEE ET AL. FIG. 5. Pathological section shows numerous irregular vascular channels lined by endothelium and separated by a fibroconnec-tive tissue stroma. silent, showing no staining or abnormal vasculature ( 2). The remainder of cases show a typical tumor blush that often has a flecked appearance associated with pooling of contrast in small collections or lakes ( 3). This appearance is apparently caused by the reduced blood flow throughout the lesion ( 2,8,9). The most commonly identified feeding arteries are the artery of the inferior cavernous sinus, the meningohypophyseal trunk, the middle meningeal artery, and the accessory middle meningeal artery. In our patient, the angiographic appearance, like the MR imaging, was thought to be most consistent with a meningioma. Cavernous sinus hemangiomas must be differentiated from the two most common benign intra-cavernous neoplasms: meningiomas and schwannomas. Indeed, most of the reported cases of in-tracavernous hemangioma were thought to be meningiomas until surgery was performed ( 1,5,8,10). As noted above, the presence on CT scanning of calcium within the lesion and hyperostosis of surrounding bone are features that strongly suggest the diagnosis of meningioma. Lack of a tumor blush on angiography favors a diagnosis of schwannoma as does the presence of an enlarged foramen ovale or foramen rotundum ( 3,11). The MR features of meningiomas, schwannomas, and intracavernous hemangiomas are quite similar, however, and may not permit differentiation among these lesions. In many cases of intracavernous hemangioma, the correct diagnosis cannot be made until a biopsy of the lesion is performed and frozen sections are obtained that show the characteristic pathologic features of the lesion. The correct diagnosis is crucial, since attempted removal of cavernous sinus hemangiomas may be associated with a substantial morbidity and mortality, primarily related to severe uncontrollable hemorrhage ( 3). Linskey and Sekhar reviewed the literature in 1992 and reported on 52 operative cases. Of these 52 cases, bleeding was described as a " problem" in 21 cases ( 42%), " massive or severe" in 15 cases ( 29%), and severe enough to require discontinuation of surgery in 3 cases ( 6%). In addition, these authors reported the results of cranial neuropathies in 27 patients after surgery. Cranial neuropathies worsened after surgery in 12 patients ( 44%), remained the same in 4 patients ( 15%), and improved in 11 patients ( 41%) ( 3). Removal of the lesion may require preoperative balloon occlusion of the ipsilat-eral internal or external carotid artery or even preoperative radiation therapy. There have been several reports that cavernous hemangiomas may be radiosensitive ( 5). Several cases treated with conventional radiotherapy have been reported ( 3,5). Indeed, our patient was treated with conventional fractionated radiotherapy of 4,500 cGy with gradual improvement of her signs and symptoms. There is, however, no long-term prospective data regarding the efficacy of this treatment. The potential role of stereotactic radiosurgery in these tumors has yet to be clearly defined ( 5). Nevertheless, the physician dealing with these lesions must be aware of all treatment options. REFERENCES 1. Goto Y, Yamabe K, Aiko Y, et al. Cavernous hemangioma in the cavernous sinus. Neurochirurgia 1993; 36: 93- 5. / Neuro- Ophthalmol, Vol. 15, No. 4, 1995 CAVERNOUS SINUS HEMANGIOMA 229 2. Katayama Y, Tsubokawa T, Miyazaki S, et al. Magnetic resonance imaging of cavernous sinus cavernous hemangiomas. Neuroradiology 1991; 33: 118- 22. 3. Linskey ME, Sekhar LN. Cavernous sinus hemangiomas: a series, a review, and an hypothesis. Neurosurgery 1992; 30: 101- 8. 4. 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