|Year : 1977 | Volume
| Issue : 2 | Page : 10-16
Primary orbital tumours
K Nath, R Gogi
M. U. Institute of Ophthalmology, Gandhi Eye Hospital, Aligarh, India
M. U. Institute of Ophthalmology, Gandhi Eye Hospital, Aligarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Nath K, Gogi R. Primary orbital tumours. Indian J Ophthalmol 1977;25:10-6
Tumours of the orbit although not rare, are still not that common so that any institution may collect and study systematically a large series of representative cases. Their scarcity is further amplified because of a wide spectrum of tissues within the orbit and every lesion usually gives rise to only one sign, that is proptosis. From India Das, Mohan et al and Gogi had rcported their series of 108, 68 and 91 primary orbital tumours respectively, where clinicopathological correlation was wantting. In the present study of one hundred and twenty consecutive histologically proved cases of primary orbital tumours clinicopathological correlation has also been attempted.
Important series of orbital tumours belong to Dandy Pfeiffer, Pfeiffer and Nichell, For rest, Craig and Gogela, Ingalls, Das, Reese, Mohan et a1 and Silva. In addition there are numerous reports concerning details of individual small tumour groups. to,,,,,, to ,,,.
| Material and Methods|| |
The present work includes study of one hundred of twenty cases of primary orbital tumours at M.U. Institute of Ophthalmology and Gandhi Eye Hospital, Aligarh. Each case was thoroughly interrogated and examined. Proptometry was done in various directions and radiological, haematological and biochemical investigations were carried out. In all cases either a surgical excision or a needle biopsy was done to establish a histopathological diagnosis, and tumours were classified on the basis of their histological origin. Thereafter an attempt was made to correlate the clinical findings with the histological origin.
| Results|| |
Amongst one hundred and twenty cases there were one hundred and three benign (85.81%) and seventeen malignant neoplasnis (14.17%), affecting seventy male and fifty females. Number and incidence of various groups of orbital tumours is shown in [Table - 1]. Amongst the new growths of the orbit, pseudotumours (28 cases) were the commonest, followed by dermoids (26 cases), vascular (19 cases). optic nerve (14 cases), mesenchymal (12 cases), peripheral nerve (11 cases) and epithelial tumours (10 cases), in that order. Complete break up of individual tumours in each group is tabulated in [Table - 2],
The age of the patients having primary orbital tumours varied from I to 69 years although they were more common during the first two decades (69 cases). Incidence of various tumours in different decades of life is tabulated in [Table - 3].
| Symptoms|| |
Passive protrusion of the eyeball was the commonest (94 cases) symptom, the duration of which varied from four weeks in malignant tumours to eighteen months in pseudotumours. Cases of dermoids presented only with a swelling round about the orbit since early childhood. The diplopia (36 cases) and pain in and around the orbit (37 cases), were the commonest complaints followed by diminution of vision (26 cases) lacrimation (11 cases) and drooping of upper lid (10 cases). Details of symptoms in each group are shown in [Table - 4]. Diplopia was frequent in cases of pseudotumours, vascular tumours as well as in mesenchymal tumours. Pain in and around the orbit was common in cases of pseudotumours, mesenchymal and epithelial tumours.
Diminution of vision was an outstanding feature with optic nerve tumours, less commonly, drooping of the upper lid and lacrimation were also recorded. Frequency of symptoms in different tumours is shown in [Table - 4].
| Signs|| |
All cases excepting those of dermoids had proptosis. Axial proptosis was present in nine cases of optic nerve tumours, four cases of pseudotumours and two cases of vascular tumours. In the rest proptosis was eccentric depending upon the site of tumour.
The swellings were well circumscribed in all the cases of dermoids, presenting at upper outer (18 cases), upper inner (6 cases) and lower outer quardrants (one case). However, the tumours could be palpated in majority of cases. Limitation of occular movements and increased intraorbital pressure were common findings. Defective pupillary reaction (23 cases), papilloedema (11 cases), optic atrophy (3 cases) with corresponding field defects, and diminution of vision (32 cases) were the frequent signs. Lid oedema and chemosis were comparatively rare. Frequency of different signs in various groups of tumours has been summarized in [Table - 5].
| Radiological Findings|| |
Increase in the density of soft tissue shadow was a regular finding, except in cases of dermoids when fossa formation and indentation was a prominent feature. Enlargement of optic formen was present in optic nerve tumours (6 cases), and in two cases of pseudotumours. Erosion of the orbital wall was also observed in eleven cases [Table - 6]. Orbitovenography was done in seventeen cases. It showed either incomplete filling (4 cases) or dilatation of veins (3 cases) or deviation of first part of the superior ophthalmic vein when tumour was located in upper inner quadrant of the orbit (6 cases. In other four cases venograms were normal.
X-ray chest in cases of malignant tumours and pseudotumours, and X-ray of small bones in pseudotumours were normal.
| Other Investigations|| |
Haematological tests and biochemical tests in cases of pseudotumours were normal.
A general systemic examination was carried out in all the cases and opinion of physicians, surgeon and ear, nose, throat specialists were also taken in every case, but was neither significant nor helpful.
| Comments|| |
Variable incidence of primary orbital tumours has been reported by different workers in their series. Silvab reported that amongst the primary orbital tumours the pseudotumours followed by dermoids occur most frequently. Haemangioma was found to be the commonest by Ingalls and Reese. On the contrary meningioma was reported to be the commonest by Dandy and pleomorphic adenoma of lacrimal gland topped the list of primary orbital tumours in the studies of Forrest, Dass and Mohan et al. In the present study pseudotumours (23.33%) and orbital dermoids (21.64%) were the commonest primary orbital new growths. These were followed by vascular (15.85), optic nerve (11.67%), mesenchymal (10.0%) peripheral nerve (9.16%) and epithelial tumours (8.33%).
This included 103 cases of benign (85.83) and 17 cases of malignant orbital tumours (14.17%). Mohan et a1 have reported an incidence of 28% and 72% of benign and malignant tumours from a study of 68 orbital tumours. However, they did not include thirteen cases of pseudotumours in this category.
In this series primary orbital tumours are seen to be more common during the second (37 cases) and first (32 cases) decades and slightly less frequently in third fourth and fifth decades [Table - 3]. Ingallas reported the first whereas Silva reported both second and third decades as the commonest age for the orbital tumours. Proptosis was 1.4 times more common in males as compared to females.
| Clinicopathological correlation|| |
This was the commonest orbital new growth and histologically majority of cases belonged to lymphoid type (15 cases). Pseudotumours can be a cause of proptosis at any age [Table - 3] .
Apart from protrusion of eyeball, pain round about the orbit and diplopia were the presenting symptoms in most of the cases. Diminution of vision in long standing cases, lacrimation and drooping of upper lid was also present in some cases [Table - 4]. On examination, proptosis was either eccentric (24 cases) or axial (4 cases). In the later case it can well be confused with optic nerve tumours. An irregular firm smooth mass could be palpated in the majority of cases. Incrcased intraorbital pressure and limitation of ocular movements were the constant finding in all those cases. Oedema of lids and chemosis was rare [Table - 5]. No specific radiological feature can be attributed to pseudotumours [Table - 6].
This is the next common new growth of our study. It generally presented during the second decade (20 cases) though the swelling was first observed as early as 2½ years and as late, as 46 years age. Patients were worried about the gradually increasing swelling along the orbital margin. Upper outer quadrant was the most frequent site (18 cases) followed by the upper inner (6 cases) and lower outer quadrant (one case). Variable indentation of bone in X-ray was observed in all the cases.
3. Vascular new growths
There were 17 haemangiomas and two malignant tumours in the form of haemangiopericytoma and haemangioendothelioma. Benign lesions were common under the age of twenty years. Both the malignant tumours were seen at the age of two years. Protrustion of eye (19 cases) and diplopia (10 cases) were the common complaints. Rarely there was pain (2 cases). The swelling could be palpated in most cases (17 cases). Limitation of ocular movements (13 cases) due to mechanical presence of the tumour was also present. Increased intraorbital pressure which was a common feature with other orbital tumours (except dermoid) was comparatively rare with haemanglomas [Table - 5]. Radiologically increase in soft tissue shadow and rarely erosion of the adjacent orbital wall could be seen [Table - 6].
4. Optic Nerve Tumours
This included nine intraorbital meningiomas and five optic nerve gliomas. All the gliomas were seen during first decade, whereas meningiomas were recorded during third, fifth, fourth and even during first decades of life [Table - 3]. Though the classical description of optic nerve tumours goes with axial proptosis, yet it was present only in nine cases. In the other five it was eccentric due to irregularity in shape of the growth in the muscle cone. On the other hand axial proptosis could also be present in cases of pseudotumours (vide supra). Other findings, such as defective pupillary reaction, papilloedema or optic atrophy, diminution of vision and enlargement of optic foramen were also recorded in different cases [Table - 4],[Table - 5],[Table - 6].
5. Mesenchymal Tumours
A wide variety of lesions were recorded in this group, indicating thereby the rarity of such tumours in the orbit [Table - 2]. These tumours were common during first two decades of life [Table - 3]. Diplopia, increased intraobiial pressure and pain round about the orbit, limitation of ocular movements, alongwith variable degrees of lid oedema and chemosis were the chief features in this group. In this regard mesenchymal tumours can easily be mistaken for pseudotumours, except that a rapidly progressive lesion point towards the malignant nature of mesenchymal tumour.
6. Peripheral Nerve Tumours
This group consisted of neurilemorna (7 cases), neurofibroma (3 cases) and one case of plexiform neuroma. These tumours were found upto the age of fifty years but for one neurilemoma occured at the age of sixty four years. Alongwith proptosis, there was pain (4 cases), diplopia (3_ cases) drooping of upper lid (2 cases) and also complained of diminution of vision [Table - 4]. A palpable swelling (8 cases) and increased intraorbital pressure (7 cases) were the common findings.
7. Epithelial Tumours
This included five cases of pleomorphic adenomas, four cases of adenoid cystic carcinomas (Cylindroma) and one case of mucoepidermoid carcinoma. These tumours were seen during third, fourth and fifth decades of life. Eccentric proptosis and pain in lacrimal gland region and lacrimation were the common complaints [Table - 4]. The swelling in upper outer quadrant, increased intraorbital pressure, limitation ocular movements were the constant findings. Poor visual acuity in five cases was due to long standing deviation of eyeball. Radiologically, increased soft tissue shadow were present in all the cases. Widening of lacrimal fossa (5 cases) and erosion of bone in three cases of cylindroma were also noted.
| Summary|| |
The present study deals with 120 consecutive cases of primary orbital tumours recorded at M.U. Institute of Ophthalmology, Gandhi Eye Hospital, Aligarh.
All the cases were fully investigated and only histopatholcgically proved cases are included.
A wide spectrum of lesions have been described such as orbital pseudotumours, dermoids, vascular tumours, peripheral and optic nerve tumours, mesenchymal tumours and epithelial tumours from lacrimal gland.
An attempt has been made to draw out a clinico-pathological correlation in different neoplastic lession.
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