CAVERNOUS HEMANGIOMA OF THE ORBIT
Prashant P Amonkar*, Rahul A Deb*,Gayathri P Amonkar**, C S Soman***
*Departments of Microbiology and **Nephrology Bombay Hospital Institute of Medical Sciences, Mumbai.
The orbit is a common site for both metastatic and primary tumours. Direct spread from adjacent structures can also occur in primary intraocular tumours such as retinoblastoma or uveal malignant tumours primary gliomas, meningiomas and lymphoid neoplasms have also been described. Among the primary mesenchymal tumours, rhabdomyosarcoma is the commonest childhood sarcoma whereas, fibrous histiocytoma is the commonest in adults.
Haemangiomas are the relatively common benign neoplasms with capillary neoplasms occurring primarily in infants and cavernous haemangiomas primarily in adult.
We present herewith a case report of a cavernous haemangioma of the orbit. Though it has been described as a “relatively common” benign neoplasm, study of the literature reveals that the incidence is not very high.
A 40 year old male presented with proptosis of the left eye since 15-16 years. Patient gave history of some ocular surgery done four years back, the details of which were not available. On examination, there was proptosis of the left eyeball. Congestion of the eye with aphakia and a corneal opacity was seen. Scar of previous surgery was also seen. A soft mass was palpable at the outer side of the left eye. No finger counting or perception of light was present.
Computerized tomography (CT) scan showed a large homogeneously enhancing mixed density mass with thinning and remodelling of the roof and medial wall of the left orbit. Impression was a haemangioma or lymphangioma.
Fine needle aspiration cytology (FNAC) on two occasions yielded only blood. An orbital exenteration was done.
Specimen of left eyeball with surrounding soft tissue measuring 70 x 50 X 50 mm. was received. Externally, a soft tissue tumour was seen engulfing the eyeball. Optic nerve was free of tumour. On cut section the tumour was reddish brown and did not penetrate the eyeball.
Large blood filled spaces lined by flattened endothelial cells were seen (Fig. 2). The spaces were of varying sizes. Intervening stroma showed lymphoid infiltrate at places. The tumour was seen to be reaching upto the eyeball but not infiltrating it (Fig. 1). Optic nerve was uninvolved. A diagnosis of cavernous haemangioma was made.
Cavernous haemangioma has been described as “relatively common” benign neoplasm of the orbit, however the incidence of the neoplasm described in literature is not very high. It occurs in the young and middle aged and is described to be more common in females. Our case was a 40 year old male.
Fig. 1 : Tumour seen in the periorbital tissue not infiltrating the eyeball. H and E, 4X.
Fig. 2 : Cavernous spaces with blood. H and E, 40X.
With the presence of multiple blood filled spaces, the FNAC finding of repeated aspirates showing only blood was as would be expected.
Though said to be common, Harris and Jacobeic found only 66 cases over a forty year period. Maroon and Kennerdell have reported only 17 haemangiomas from among 300 cases of orbital tumours over a period of seven years. Nath et al have reported 12 cases among 120 primary orbital tumours. Gunalp and Gunduz et al. have reported 37 cases in a 30 year study.
CT scan is diagnostic and reveals a well demarcated retrobulbar tumour thinning the roof and medial wall of the orbit. The differential diagnosis lies between a haemangioma and a lymphangioma.
Hyperdensity in the precontrast scan and enhancement with contrast study have been described. Magnetic resonance imaging (MRI) has been described to be useful in the radiological diagnosis.
Angiography of the tumour will show characteristic pooling of contrast in the spaces.
Treatment of the tumour is surgical excision. Microsurgical techniques have been described where the tumour can be excised with minimal damage of the surrounding structures. The timing of the surgery is important as in later stages, once the visual loss is complete surgery is not very helpful.8 In our case, there was total loss of vision and hence an exenteration was done.
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- Harris C, Jacobeic FA. Cavernous hemangioma of the orbit. J Neurosurg 1979; 51 : 219-28.
- Maroon JC, Kennerdell JS. Tumours of the orbit. In : Wilkins RH and Rengachay SS, editors. Neurosurgery New York. McGraw Hill Co. 1985; 1 : 964-76.
- Nath K, Gopi R, Khan AA, et al. Vascular hamartoma and vascular tumours of the orbit. Ind Journ of Ophthal 1977; 25 : 18-23.
- Gunalp I, Gunduz K. Vascular tumours of the orbit. Doc Ophthalmol 1995; 89 (4) : 337-45.
- Forbes G. Radiologic evaluation of orbital tumours. In Clinical neurosurgery. Baltimore : Williams and Wilkins. 1985; 32.
- Housepain EM, Trokel SL, Jacobiec FA, et al. Tumours of the orbit. In : Youmanns JR, editor. Neurological Surgery. Philadelphia, WB Saunders. 1990; 3371-411.
- Missori P, Tarantino R, Delfini R, et al. Surgical management of orbital cavernous angiomas : Prognosis for visual function after removal. Neurosurgery 1994; 35 : 34-38.