|Year : 1963 | Volume
| Issue : 2 | Page : 38-42
Primary tumours of the orbit
Pathology Department, Institute of Ophthalmology, Calcutta, India
|Date of Web Publication||28-Jan-2008|
S P Das
Pathology Department, Institute of Ophthalmology, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Das S P. Primary tumours of the orbit. Indian J Ophthalmol 1963;11:38-42
Due to the structure and development of the orbit, primary tumours of the orbit comprise a large group of tumours. The subject has become complicated by the different names given to a single tumour e.g. mixed tumour of the lacrimal gland is also known as adenocarcinoma, cylindroma, myxochondrocarcinoma, adenoma etc. It appears more difficult since in most of the cases correct diagnosis of a specific tumour cannot be done by clinical methods of examination without the help of surgery and the histopathological examination of the specimen removed. The symptoms and signs are more general rather than specific for a particular tumour, so in most of the tumours treatment and prognosis depend largely on the histopathological report.
In most of the cases patients come to the ophthalmologist with exophthalmos and its complications. The commonest complication of exophthalmos is exposure of the cornea which later on leads to corneal ulcer. The tumour mass may press inside the orbit on the optic nerve and may produce papilloedema ophthalmoplegias and optic atrophy.
The present observations on 108 cases of primary tumours of the orbit were carried out in the Institute of Ophthalmology, Calcutta, over a period of 12 years. Only the cases where surgery and histopathological examinations were done were listed as follows:
List of Primary Tumours of the Orbit,
1. Mixed tumours of the Lacrimal gland ... 16
2. Dermoid cyst ... 15
3. Haemangioma ... 13
4. Lymphoma ... 13
5. Lymphosarcoma ... 10
6. Schwannoma ... 8
7. Undifferentiated Sarcoma ... 8
8. Glioma ... 5
9. Neurofibroma ... 5
10. Lipoma ... 5
11. Meningioma ... 3
12. Rhabdomyosarcoma ... 3
13. Fibroma ... 2
14. Angiosarcoma ... 2
Total series of tumours = 108
From the list it is obvious that some are common tumours and others that are rare. The list has been arranged in order of frequency.
The commoner tumours will be discussed in this paper.
| Mixed Tumours of the Lacrimal Gland|| |
No case of bilateral incidence was noted in the present series. The age varied from 17 years to 56 years. Proptosis was a constant symptom in all cases. The common clinical finding vas a well-localised, firm, sometimes hard mass situated in the superolateral quadrant of the orbit with nodular surface. There was displacement of the globe down and medially and limitation of ocular mobility up and out.
Histologically, both epithelial and mesodermal elements which varied in different amount took part in the tumour production.
Mixed tumours of the lacrimal gland have a great tendency to invade the adjacent bone, the extent for which is determined by X-Ray and should guide the prognosis and treatment.
These tumours may be both benign and malignant. In the present series of sixteen cases, out of the ten histologically determined benign tumours three later turned into malignancy. So histological diagnosis of the benignity of mixed tumours of the lacrimal gland is not a sure guide to the prognosis and all these tumours should be treated with a suspicion of malignancy and the patient should be kept under observation for a long time.
In the follow up of these tumours, three out of ten histologically benign cases showed recurrence and two of them died. The other one is symptom free now after deep X-Ray therapy. Out of the six histologically determined malignant cases four died clue to intracranial extension after recurrence; one is living with recurrence and the sixth is symptom free now.
| Dermoid Cyst|| |
Although dermoid cysts are congenital, symptoms start after a few years of life. The present series of fifteen cases of dermoid cysts of the orbit were noted in the second and third decades of life.
Microscopically, dermoid cysts resembled the structure of skin. The cyst wall was composed of two layers. The outer connective tissue layer contained blood vessels, hair follicles, sebaceous glands, fat, muscle etc. The inner epithelial layer varied in thickness from the usual of one to two layers of squamous epithelium to all the layers including epidermis. The cavity of the cyst contained the secretion of sebaceous and sweat glands. Sometimes hairs were noted in the cystic cavity.
Dermoids of the orbit may produce secondary adhesions to the surrounding structures of the orbit due to inflammatory changes. For this reason, sometimes great difficulty is encountered in their operative removal. In two of the present cases, slight damages to the surrounding structures of the orbit were due to this reason.
The usual treatment of complete operative removal was adopted in the present series of cases and no recurrence was noted in any of the cases.
| Haemangioma|| |
The tumours are probably congenital and the majority of the present cases were noted at the lower age group. The youngest one recorded was at the age of four years and the oldest one was fifty years. All the present fourteen cases were of cavernous variety. In none of the cases there were co-existent angiomas of the skin. The compressability of the tumour and the non-interference of the ocular mobility helped to a great extent in the clinical diagnosis.
All the tumours were well-encapsulated and the capsule consisted of collagenous and elastic fibres. The tumours were composed of large blood spaces lined by endothelium, bound together by connective tissue in the form of trabeculae. Secondary changes in the form of sclerosis (sclerosing haemangioma) were noted in three of the present fourteen cases.
| Lymphoma|| |
None of the present thirteen cases of lymphoma of the orbit were primarily associated with typical leucaemic blood picture. Although there is no particular age incidence, majority of the present series of cases occurred above thirty-five years of age. Six of the thirteen cases had bilateral incidence. Proptosis and dimness of vision were the two constant complaints. Histologically, the tumours showed uniform mature lymphocytes. Several typical follicles were also seen occasionally. Seven of the thirteen patients could be followed up. Two of them later on showed typical leucaemic blood picture and died. Irradiation gave dramatic response as the tumours are highly radiosensitive. One patient refused irradiation after biopsy and returned after two and a half years with generalised lymphomatous deposits throughout the body. Four patients after irradiation therapy are doing well at present.
| Lymphosarcoma|| |
Lymphosarcoma is the malignant variety of lymphoma. In the present series of ten lymphosarcoma of the orbit, ages ranged from 2 years to 6o years. Seven of these tumours showed localised lesions in the orbit, but in the other three there were evidences of generalised disease at the first visit of the patient to the hospital. Histologically, the tumours were composed of dense collection of lymphatic cells with different stages of maturations. Mitotic figures were common and the cells showed marked malignant character. Many of the tumours had great tendency to invade the surrounding structures.
The tumours possess a poor prognosis and irradiation only prolongs the life of the patient. Three patients who came with generalised metastasis died within two months to one and a half years. Two patients with irradiation are doing well, one and a half years, and two years after operation respectively. Two patients died from generalised metastasis. No follow-up data was available in the other three cases.
| Schwannoma|| |
Schwannoma of the orbit is a well encapsulated, localised, benign, nerve sheath tumour with characteristic histological picture, but without any specific clinical picture.
In the present eight cases the youngest patient was twelve years old and the oldest one was firty-five years old. Most of the tumours were anteriorly situated in the orbit and could be palpated through the lids.
On histopathological examination, the tumours were found to be firm, encapsulated with irregular surfaces. The tumours were composed of plenty of spindle cells, collagenous fibres and numerous blood vessels. Degeneration in the form of calcification, necrosis and cystic change were present in different tumours in varying proportions. The characteristic arrangement of palisading of the nuclei of the elongated cells were the constant feature in all cases. There was no sign of malignancy in any of the cases.
The treatment adopted was complete operative removal and no recurrence was observed.
| Summary|| |
A series of rob cases of primary tumours of the orbit were recorded in the Institute of ophthalmology, Calcutta, over a period of twelve years. Although the list of such tumours is large the common tumours come to a small series which have been commented on. In most of the cases, diagnosis, prognosis, and treatment depended upon the histopathological report.
| Acknowledgement|| |
I am indebted to Prof. K. L. Son, Director, Institute of Ophthalmology, Calcutta for giving me encouragement and permission for publishing this paper. I am also grateful to Prof. S. N. De and Dr. P. K. Sarkar of Medical College, Calcutta for giving Ire valuable suggestion in this paper.
| References|| |
Benedict, W. L., (1949), Amer, J. Ophthal. 32, 763.
Benerman, V. A. and Tabor, G. L., (1958), Amer, J, Ophthal 46, 501.
Duke-Elder, W. S. : Text Book of Ophthalmology, St. Louis, Mosby, 1952. V-5., P-5530.
Forrect, A. W., (1949), Arch. Ophthal. 41, 198.
O'Brien, C. S. and Leinfelder, P. J., (1935), Amer, J. Ophthal. 18, 123.
Reese, A. B., (1956), Amer. J. Ophthal. 41, 3.
Reese, A. B., (1935), Arch. Ophthal. 14, 41 .
Reese, A. B : Tumours of the Eye, Newyork, Hoeber, 1950.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]