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Year : 1977  |  Volume : 25  |  Issue : 1  |  Page : 18-23

Vascular hamartoma and vascular tumours of orbit

1 A. M. U. Institute of Ophthalmology, Aligarh (UP), India
2 Department of Pathology, J.N. Medical College, Aligarh (UP), India

Correspondence Address:
K Nath
A.M.U. Institute of Ophthalmology, Aligarh (U. P.)
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Source of Support: None, Conflict of Interest: None

PMID: 612586

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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

How to cite this URL:
Nath K, Gogi R, Khan A A, Hameed S. Vascular hamartoma and vascular tumours of orbit. Indian J Ophthalmol [serial online] 1977 [cited 2023 Jun 6];25:18-23. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1977/25/1/18/34595

Table 1

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Table 1

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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 [35] . Hogen and Zimmerman [17] 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 [1] . It is more commonly seen during the first year of life [16] 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 [23],[39],[44] . This may be associated with haemangioma of the lids and cheeks [8] . It increases when the patient cries or when pressure is applied upon the jugular vein or during menstruation [20] and even preg­nancy [35],[45] . Haemangiomas have been described both in the series of oribital tumours [7],[9],[10],[26],[29],[35],[38] and as isolated cases [2],[3],[5],[15],[21],[22],[27],[32],[37] .

Haemangiopericytoma is a very rare humour of the orbit [17] . Very few cases are on record in the literature. [6],[11],[13],[14],[24],[25],[36],[40],[43] This tu­mour can occur at any age [10] and has no rela­tionship with sex. It arises from the pericytes of Zimmerman and is a true neoplasm from the blood vessels. [17]

In 1943, the term shaemangioendothelioma was suggested by Stout [41] 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 [28] and usually occurs in infants with no specific sex preponderance. [17]

  Material and Methods Top

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 Top

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.

1. Haemangiomas

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 Top

In this group one case of malignant hae­mangiopericytoma and the other of malignant haemangioendothelioma were found.

Case I

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.

Case II

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 Top

A haemangioma reproduces the whole stru­cture of the vessel wall and is considered to be a development anomaly rather than a neo­plasm. [2],[44]

Haemangioma is the most common primary orbital growth which can be of capillary type or cavernous type [4],[9],[33],[34] or a venous type. [17] In the present study, there were seventeen cases of vascular hamartomas and that consti­tutes 15.84% of all primary obrital tumours. [31] 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. [12]

There were twelve cases of cavernous hae­mangiomas, three of capillary and one of venous haemangioma. According to Ingalls [19] and Hogan and Zimmerman [17] 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 [17] suggested that mali­gnant haemangiopericytoma does not arise from its benign counter part. Stout and Lattes [42] 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 [17] . 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. [31] This points towards the rarity of this tumour.

In a series of 222 intraorbital tumours, Forrest [9] found only one case of haemangio­endothelioma. On other hand, Iliff [18] found three cases in a series of 88 primary orbital tumours and Mortada [28] four in a series of 130 cases. Mukherji [30] and Kojima at al [22] have also reported one case each.

  Summary Top

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 Top

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 Top

Abernethy, 1810, Surgical observations, London. Quoted by, Duke Elder, S., (1952).  Back to cited text no. 1
Albrecht, E., 1904, Uber Hamartoma, Ueerh, Dtsch. Path. Ges., 7, 153.  Back to cited text no. 2
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Birch-Hirschfeld, A. 1930, Graefe Sernisch Hand­bitch der gesamten AugLnheilkunde, 2nd Ed. Band 9, abt. 1, teil 1. Springer, Berlin.  Back to cited text no. 4
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Duke Elder, S., 1952, Text Book of Ophthal., Vol. V, P. 5565, Henry Kimpton. London.  Back to cited text no. 8
Forrest, A.W., 1949, Arch. Ophthal., 41, 198.   Back to cited text no. 9
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Iliff, C.E., 1957, Arch. Ophthal„ 65, 636.  Back to cited text no. 18
Ingalls, R.G., 1953, Tumours o, the orbit & Allied pseudotumours, Thomas springfield iii, Blackwell Scientific Publication, Oxford.  Back to cited text no. 19
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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]

  [Table - 1]


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