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Year : 1999  |  Volume : 47  |  Issue : 3  |  Page : 248-9

Cavernous sinus haemangioma with hereditary multiple exostoses.






How to cite this article:
Bhatoe H S. Cavernous sinus haemangioma with hereditary multiple exostoses. Neurol India 1999;47:248


How to cite this URL:
Bhatoe H S. Cavernous sinus haemangioma with hereditary multiple exostoses. Neurol India [serial online] 1999 [cited 2020 Jan 24];47:248. Available from: http://www.neurologyindia.com/text.asp?1999/47/3/248/1600



Intracranial extra-axial cavernous haemangiomas are benign, well demarcated neoplasms that arise within the dural sinuses.[1] Such tumours arising from the cavernous sinus are distinctly rare.[2] One such tumour occurring in a patient with hereditary multiple exostoses (HME) is reported.

A 35-year-old male was admitted with one-month history of right-sided ptosis and mild intermittent headache. Clinically, he had multiple exostoses over the long bones in all four limbs, and significant cafe au lait spots. There was complete ophthalmoplegia with ptosis on the right side, with preserved vision. In addition, he had sensory impairment over the ophthalmic division of the trigeminal nerve. There was a strong family history of HME. Computed tomography (CT) of the brain showed dumbbell shaped, well-defined, non-calcified lesion arising from the right cavernous sinus. The mass enhanced densely and uniformly with intravenous contrast [Figure 1]. Right carotid angiography, done to assess the vascularity of the tumour, showed an avascular extraaxial temporal region mass. Radiographs of the long bones confirmed HME [Figure 2]. Patient was taken up for surgery with a suspicion of cavernous sinus haemangioma. The tumour was exposed by right frontotemporal craniotomy. Sphenoid wing was drilled for additional exposure. Dural opening revealed smooth surfaced, well defined dark, vascular extra-axial mass arising from the cavernous sinus. The mass was excised leaving its attachment with the cavernous sinus. Postoperative period was uneventful. Diagnosis of cavernous sinus haemangioma was confirmed on histopathology. Extra-axial cavernous sinus haemangiomas are rare lesions that usually present with mass effect, including headache and cranial nerve palsies.[3] In an extensive review, after carefully excluding duplicate citations, Linskey and Sekhar[4] collected 53 confirmed cases of cavernous sinus haemangiomas published between 1943 and 1991. Suzuki et al[2] reported seven more cases. Cavernous sinus haemangiomas have an overwhelming predilection for women. In fact, of the 39 cases in which the sex of the patient is identified, only three occurred in men.[4] Majority of the reported cases have been reported in the Japanese.[1],[2],[4]

On CT, characteristically, these tumours are not calcified, and enhance densely and homogeneously with intravenous contrast. Carotid angiography reveals an avascular extra-axial temporal region mass. Magnetic resonance imaging is the most useful study to diagnose a haemangioma. Cavernous sinus haemangiomas are isointense on T1 weighted images and markedly hyperintense (like the CSF) on T2 weighted images.[4] The lesions that have similar appearances on MRI are angioblastic meningiomas and neurilemmomas,[5] both extremely rare in the cavernous sinus.

The association of cavernous sinus haemangioma and HME has not been reported earlier. HMF is a known autosomal dominant genetic disorder and it is likely that this patient belongs to a genetic syndrome occurring due to fresh mutations.
 

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1.Meyer FB, Lombardi D, Scheithauer B et al : Extraaxial haemangiomas involving the dural sinuses. J Neurosurg 1990; 73 : 187-192.  Back to cited text no. 1    
2.Suzuki Y, Shibuya M, Baskaya MK et al : Extracerebral cavernous haemangiomas of the cavernous sinus in the middle fossa. Surg Neurol 1996; 45 : 123-132.  Back to cited text no. 2    
3.Harper DG, Buck DR, Early DO : Visual loss from cavernous haemangiomas of the middle cranial fossa. Arch Neurol 1982; 39 : 252-254.  Back to cited text no. 3    
4.Linskey ME, Sekhar LN : Cavernous sinus haemangioma : A series - a review and an hypothesis. Neurosurgery 1992; 30 : 101-108.  Back to cited text no. 4    
5.Watabe T, Azuma T : T1 and T2 measurement of meningiomas and neuromas before and after Gd-DTPA. AJNR 1989; 10 : 463-470.  Back to cited text no. 5    

 

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Online since 20th March '04
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