|Year : 1977 | Volume
| Issue : 1 | Page : 18-23
Vascular hamartoma and vascular tumours of orbit
K Nath1, R Gogi1, AA Khan2, S Hameed2
1 A. M. U. Institute of Ophthalmology, Aligarh (UP), India
2 Department of Pathology, J.N. Medical College, Aligarh (UP), India
A.M.U. Institute of Ophthalmology, Aligarh (U. P.)
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nath K, Gogi R, Khan A A, Hameed S. Vascular hamartoma and vascular tumours of orbit. Indian J Ophthalmol 1977;25:18-23
A vascular hamartoma generally reproduces the whole structure of the vessel wall, and a true vascular neoplasm arises from the individual tissue of the wall of the blood vessel  . Hogen and Zimmerman  classified the orbital angioma as benign and malignant. Benign group includes capillary haemangioma, cavernous haemangioma, haemangiopericytoma and haemangioendothelioma. The malignant group includes malignant haemangiopericytoma, malignant haemangioendothelioma and Kaposi's sclerosing angiosarcoma.
The first case of haemangioma of the orbit was published by Abernethy in 1810  . It is more commonly seen during the first year of life  and there is no sex relationship. It causes slowly progressive proptosis. Quite often a haemangioma has a period of rapid growth concomitant with the growth of the child and then in the majority it regresses spontaneously or at least shows evidence of regression by the age of fifteen years ,, . This may be associated with haemangioma of the lids and cheeks  . It increases when the patient cries or when pressure is applied upon the jugular vein or during menstruation  and even pregnancy , . Haemangiomas have been described both in the series of oribital tumours ,,,,,, and as isolated cases ,,,,,,,, .
Haemangiopericytoma is a very rare humour of the orbit  . Very few cases are on record in the literature. ,,,,,,,, This tumour can occur at any age  and has no relationship with sex. It arises from the pericytes of Zimmerman and is a true neoplasm from the blood vessels. 
In 1943, the term shaemangioendothelioma was suggested by Stout  to designate the tumour characterized by the proliferation of endothelial cells of the lumen of blood vessels. This is a very rare tumour of the orbit  and usually occurs in infants with no specific sex preponderance. 
| Material and Methods|| |
In a study of one hundred and twenty cases of primary orbital tumours which were examined, operated and histologically confirmed at A.M.U. Institute of Ophthalmology, Aligarh, seventeen cases of vascular hamartoma and two of vascular tumours were recorded.
| Observations|| |
Nineteen cases of vascular new growths of the orbit affected twelve males and seven females in a consecutive study of 120 primary orbital tumours. The age varied from I to 48 years, with the highest incidence during the first decade (10 cases). There were seven teen haemangiomas and two malignant tumours in the form of haemangiopericytoma and haemangioendothelioma.
Clinically there xas history of unilateral proptosis of gradual onset in all the seventeen cases. In four cases there were associated haemangiomas in the upper lid as well. In one case there was a mass bulging through lower lid and the eyeball was pushed up [Figure - 1].
There was history of diplopia (10 cases) and pain in and around the orbit (2 cases).
On examination the swellings could be palpated in fifteen cases. Due to the mechanical presence of the tumour, limitation of the ocular movements (11 cases) was also present. Increased intraorbital pressure was detected in three cases only.
On gross examination the tumour masses were encapsulated [Figure - 2], and the size was variable (approximately 1.0 x 1.0 cm to 3.0 x 3.5 cm). They were either round or oval and were spongy in consistency. The cut surface showed numerous spaces of different sizes filled with the blood [Figure - 3]. The microsopic examination revealed the following three types of histological picture:
(i) In four cases there were multiple small vascular channels lined by a single layer of endothelium. In the lumen, the erythrocytes were present [Figure - 4] and a diagnosis of capillary haemangioma was made. (ii) In tweleve cases, there w ere large irregular spaces containing erythrocytes and were lined by flattened endothelial cells. 'I he spaces intercommunicated with each other and showed papillary infoldings of the wall. In between the spaces fibrous septa were present [Figure - 5]. In two cases the tumour was found to invade the orbital fat. All these cases belonged to cavernous type of haemangiomata. (iii) In one case smooth muscle fibres were noted in the walls of the vascular spaces and thus constituted a venous haemangioma.
| Vascular Tumours|| |
In this group one case of malignant haemangiopericytoma and the other of malignant haemangioendothelioma were found.
A female child aged two years, presented with rapidly progressive proptosis of the eyeball for three months. Plain anterio posterior x-ray of the orbit showed marked increase in the size of orbital cavity [Figure - 6]. The tumour mass was found to infiltrate all the orbital tissues and excenteration of the orbit was carried out. On gross examination, an irregular softish tumour mass measuring 4.5.x 3.0 x 2.0 cm (approximately) was seen. Under the microscope, it consisted of rich vascular tissue showing numerous small capillary lumens which appeared to be surrounded by two or three layers of round or oval pale staining cells with prominent vesicular nuclei. On staining with haematoxylin and eosin, it was difficult to decide whether these round or oval cells were endothelial cells or pericytes. With silver impregnation, the reticulin fibres appeared black and these cells were present outside the reticulin network. Hence these cells were pericytes and the diagnosis of haemangiopericytoma was confirmed.
A female child aged two years was admitted with the complaints of gradually increasing proptosis for three months. At the time of operation, the tumour mass was seen to infiltrate the orbital tissues and the excenteration was done. It was an irregular, small fleshy mass measuring 2.0 x 1.0 x 1.0 cm and the cut surface was dark brown in colour.
The microscopic section, when stained with haematoxylin and eosin showed the presence of streak like spaces lined by several layers of atypical endothelial cells with large hyperchromatic nuclei. After silver impregnation the cells were found to be present inside the reticulin network [Figure - 7], and the diagnosis of haemangioendothelioma was confirmed. Histological diagnosis of these growths are shown in [Table - 1].
| Discussion|| |
A haemangioma reproduces the whole structure of the vessel wall and is considered to be a development anomaly rather than a neoplasm. ,
Haemangioma is the most common primary orbital growth which can be of capillary type or cavernous type ,,, or a venous type.  In the present study, there were seventeen cases of vascular hamartomas and that constitutes 15.84% of all primary obrital tumours.  Such lesions are more common in the younger age group and were responsible for 23.63% of primary orbital new growths under the age of fifteen years. 
There were twelve cases of cavernous haemangiomas, three of capillary and one of venous haemangioma. According to Ingalls  and Hogan and Zimmerman  both elements of cavernous and capillary haemangioma, in varying proportions, can be seen in the same tumour. In none of our cases this finding could be corroborated.
Amongst the vascular tumours, one case each of haemangioendothelioma and haemangiopericytoma were examined. On the basis of histopathological findings alone, it was not possible to decide whether these tumours were beingn or malignant. However. both the tumours were diagnosed as malignant because the tumours extensively infiltrated the adjacent orbital tissues and in the case of haemangiopericytoma there was enlargement of the orbital cavity in the x-ray. Hogan and Zimmerman  suggested that malignant haemangiopericytoma does not arise from its benign counter part. Stout and Lattes  felt that there is no reliable histological criteria to differentiate the benign and malignant varieties of haemangiopericytoma. Haemangioendothelioma may appear invasive and cellular and yet behave clinically as a benign tumour  . Thus the invasive nature of the tumour in our case further supports the malignat nature of the tumour. In a series of 120 cases there was only one case each of haemangioendothelioma and haemangiopericytoma.  This points towards the rarity of this tumour.
In a series of 222 intraorbital tumours, Forrest  found only one case of haemangioendothelioma. On other hand, Iliff  found three cases in a series of 88 primary orbital tumours and Mortada  four in a series of 130 cases. Mukherji  and Kojima at al  have also reported one case each.
| Summary|| |
1. Seventeen cases of vascular hamartomas and two of vascular tumours in 120 consecutive cases of primary orbital tumours has been reported.
2. There are seventeen benign and two malignant tumours.
3. The benign haemangiomas consist of cavernous type (12 cases), capillary type (4 cases), and venous type (I case). The malignant group includes one case each of haemangiopericytoma and haemangioendothelioma.
4. Histopathological appearance of these new growths has been described in detail.
5. The rarity of the malignant types is discussed in different series.
| Acknowledgement|| |
We are thankful to Mr. A.Y. Khan, Senior technician of Institute, for processing the tissue for histopathological examination. We are also grateful to Mr. U.C. Gupta, senior photo-technician of Institute for carrying out photography work for us.
| References|| |
Abernethy, 1810, Surgical observations,
London. Quoted by, Duke Elder, S., (1952).
Albrecht, E., 1904, Uber Hamartoma, Ueerh, Dtsch. Path. Ges., 7,
Bernadezykowa, A. and Kwaskowski, A., 1961, Klin. Oczna.,
Birch-Hirschfeld, A. 1930, Graefe Sernisch Handbitch der gesamten AugLnheilkunde,
2nd Ed. Band 9, abt. 1, teil 1. Springer, Berlin.
Bisaria, K.K. and Misra, U.C., 1968, Brit. J. Ophthal., 52,
Carlon, G. and Sacchi, A.G., 1967, Ann Ottal.,
Dass, S.P., 1963,.7. All India Ophthal. Soc., 11,
Duke Elder, S., 1952, Text Book of Ophthal.,
Vol. V, P. 5565, Henry Kimpton. London.
Forrest, A.W., 1949, Arch. Ophthal., 41,
Foster, J., 1955, Ann Roy. Coll. Surg., 17,
Fox, S.A., 1955, Amer J. Ophthal., 40,
Gogi, R. and Nath. K., 1976, East. Arch. Ophthal.,
Goodman, S.A., 1955, Amer. J. Ophthal., 40,237.
Heney, R.F., 1964, Arch. Ophthal., 71,
Hobbs, H.E., 1961-a, Brit. J. Ophthal., 45,
Hobbs, H. E., 1961-b, Trans. Ophthal. Soc. U. K.,
Hogan, M. J. and Zimmerman. L. E., 1962. Cphthalmic Pathology,
An Atlas and Textbook 2nd Ed. P. 741. W.B. Saunders Co., Philadelphia & London.
Iliff, C.E., 1957, Arch. Ophthal„
Ingalls, R.G., 1953, Tumours o, the orbit & Allied pseudotumours,
Thomas springfield iii, Blackwell Scientific Publication, Oxford.
Kafka, 1928, Lijecnicki Vjesnik,
Katsourakis, N. and Roussos, J., 1963, Bull. Soc. hellen. Ophthal. 31, 7.
Kojima, K., Okochi, Y. and Yamada, J. 1969; Folia Ophth. Jap., 20,
Lister, W.A., 1938, Lancet., 1,
Macoul, K.L.. 1968, Amer J. Ophthal.,
Maria, D.L., Apte, P.V. and Joshi, B.G., 1971, Orient. Arch. Ophthal. 9,
Menning, H., 1965, Wiss. Z. Humboldt Univ., 6,
Mortada, A., 1962, Brit. J. Ophthal.,
Mortada, A., 1963, Brit. J. Ophthal., 47,
Mohan, H. Sen, D.K. and Gupta, D.K., 1968, Orient. Arch. Ophthal., 6,
Mukherji, R., 1967, Indian J. Ocular Path., 1, 62.
Nath, K. and Gogi, R., 1976, Proc. Vi Afro-Asian
Cong. (In press)
Nano, H., Aragon, F. and Perez, H., !967, Arch. Oftal B. Aires,
Reese, A.B., 1941, Amer. J. Ophthal., 24,386.
Reese. A.B., 1941, Amer. J. Ophthal.,
Reese, A.B., 1963, Tumours of the Eye.,
Ed. 2, pp. 368, 408, 409, Hoeber, N.Y.
Reich, H., 1972, Klin Mbl. Augenheilk.,
Rowbotham, G.P. and Little, E., 1966, Brit. J, Ophthal.,
Silva D., 1968, Amer. J. Ophthal.,
Simpson, J.R., 1959, Lancet., 2,
Spaeth, E.B. and Valdes-Dapona, A., 1958, Arch. Ophthal., 60,
Stout, A.P., 1943, Ann. Surg,,
Stout, A.P. and Lattes, R., 1967, Tumours of soft tissue.
Fascicle 1, Second Series of the atlas of Tumour Pathology, Armed Forces Institute of Pathology.
Stout, A.P., 1949, Cancer, 2,
Willis, R.A., 1958, The borderland of embryology and pathology, p.
342, Butterworth & Co. Ltd. London.
Zauberman, H., Feinsod M, 1970, Acta Ophthal.,
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]
[Table - 1]