Year : 1973 | Volume
: 21 | Issue : 4 | Page : 161--165
Primary tumours of the orbit causing unilateral proptosis- (Part I-incidence and clinical features)
Hari Mohan, DK Sen
Department of Ophthalmology, Irwin Hospital, New Delhi, India
Department of Ophthalmology, Irwin Hospital, New Delhi
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Mohan H, Sen D K. Primary tumours of the orbit causing unilateral proptosis- (Part I-incidence and clinical features).Indian J Ophthalmol 1973;21:161-165
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Mohan H, Sen D K. Primary tumours of the orbit causing unilateral proptosis- (Part I-incidence and clinical features). Indian J Ophthalmol [serial online] 1973 [cited 2019 Nov 22 ];21:161-165
Available from: http://www.ijo.in/text.asp?1973/21/4/161/34622
The structures and development of the orbital contents are such that a wide variety of tumours may arise in this situation. However, the data presented by various authors as to the incidence of various lesions are not comparable since dissimilar criteria have been used by them in the selection of cases. The difference in incidence can also be attributed by variations in approach to the subject.
The present series comprise a total of 91 consecutive cases of unilateral proptosis of neoplastic origin seen in the Eye O.P.D. of Irwin Hospital. Primary tumours of the orbit constituted 14 cases (19.3%). These tumours have been classified according to their origin [Table 1] and will be presented in the order of this grouping.
Proptosis was a constant finding in all the cases. In some of them proptosis was extreme and gave rise to complications like exposure of the cornea leading to corneal ulcer. In one case the forward protrusion of the globe was so extreme as to give rise to dislocation of the globe. Besides proptosis the clinical features included decreased vision, disturbance of ocular mobility, palpable mass, fundus changes (papilloedema, optic atrophy, traction lines) and field changes.
A brief account of the lesions with respect to findings in these 54 cases is given below:
MESENCHYMAL TUMOURS (28 cases)
Haernangiomas: Of the 10 cases in this series 4 were diffuse in character (infantile type) and 6 were well encapsulated (adult type). Two of the infantile types had associated skin haemangiomas and one had a bluish mass in the roof of the mouth. The proptosis was greater than 10 mm. in five cases. Three of the adult cases had a discrete palpable mass on clinical examination. One case had multiple haemangiomas all over the upper half of the body. Another case where the haemangioma was situated deep in the muscle cone had tortuous and dilated episcleral vessels. No fundus changes were found except in two cases which had retinal striae. It is interesting to note that the tumour did not interfere with ocular movements in any of the cases.
Fibroma (1 case)
The tumour occured in a female at the age of 40 years. It was situated anteriorly in the lower portion of the orbit on the left side, pushing the lower lid forwards. On palpation it felt firm, attached to the deeper structures but free from the bone.
Rhabdomyosarcoma (5 cases)
Of the 5 cases 3 occured between the ages of 16 and 50 years. All excepting 2, manifested as a very rapidly enlarging mass. Proptosis was eccentric in 3 cases and was between 8 and 12 mm. Optic disc was completely atrophic in 2 cases.
Undifferentiated Sarcoma (3 cases)
They had no special clinical features excepting one case where necrosis of the tumour excited an acute inflammation.
Reticulo-endothelial Tumours (8 cases)
Their ages ranged from 10 to 49 years. In 3 of them orbital involvement was late during the course of an already generalised lymphomatous disease. In 2 cases proptosis was the presenting feature of the disease but by the time the patients reported to the hospital the condition had progressed to involve the other parts of the body. Only in the remaining 3 cases the affection was limited to the orbit. Proptosis was a constant finding but in one case it was so marked that the globe was almost dislocated. There was a palpable soft to firm mass in 5 cases, in 2 of which the mass appeared to extend forwards from deep in the orbit. None of them were associated with typical leukaemic blood picture. Papilloedema was present in 2 cases.
EPITHELIAL TUMOURS (15 cases)
Lacrimal Gland Epithelial Tumours Seven of the 11 cases were between 16 and 50 years and four were over 50 years of age. In 4 cases pain was a feature because of involvement of the adjacent periosteum. In 5, proptosis was the presenting complaint and in 4, presence of a noticeable mass was the initial symptom. Displacement of the globe down and in and limitation of ocular rotation up and out to a varying degree were constant findings excepting one case where the displacement was downwards and forwards. A firm to hard mass could be palpated in all the cases, seven of which appeared localized. In four of the cases, surface of the palpable mass was nodular. Orbital crest was found thickened in 3 cases. In one case there was pressure type of optic atrophy due to backward extension of the tumour.
Carcinoma of Unknown Origin (4 cases)
All the cases were between 35-50 years. All of them reported late with a fungating lesion and it was not possible to establish the site of origin. The globe was destroyed in every case.
NEURAL TUMOURS (11 cases)
Neurofibroma: In the two cases in this series the condition occured at the anterior part of the orbit as an isolated lesion. One of the patients reported to the hospital with the history of recurrent attacks of pain in and around the eye over a period of 2 years. Cafe-au-lait pigmentations were present in one case.
Neurilemmoma (4 cases) There were no characteristic clinical features except that they were encapsulated and occured in the orbit as an isolated lesion. One of the 4 cases, aged 14 years, presented with the clinical features of retrobulbar neuritis and the correct diagnosis was established when the patient reported later again after 3 years with the full blown picture of a space occupying orbital lesion (MOHAN,AND SEN  ). Two of the tumours were so anteriorly situated in the orbit that they could be palpated through the lids. Signs indicating a relation to von Recklinghausen's disease were not present in any of the cases.
Glioma of Optic Nerve: All the 5 cases were above 15 years of age excepting one. They usually presented with the history of gradual diminution of vision which was early and profound and %or unilateral proptosis. In this series in 2 cases the proptosis was axial, in 2 cases the globe was displaced forwards, downwards and outwards, and in one case the globe was displaced forward, upwards and outwards A firm mass could be felt in 2 cases by pushing the index finger between the globe and the lateral wall of the orbit. Pressure type optic atrophy was seen in two cases. In one case lids were oedematous and the conjunctiva was chemosed and congested (MOHAN AND SEN  ). Of the 5 cases 2 were intraorbital, 2 retroorbital and 1 chiasmal.
Frequency of lesions in some important similar series as reported, differ widely. It would be seen that the incidence depended on which speciality group had conducted the study. In a radiological series at the Institute of Ophthalmology, New York -REESE,  meningiomas were the commonest neoplasm (7%) which was followed by haemangiomas (6%). In a histologic series (cited by REESE  ) haemangioma was most commonly present (15.2%) which was followed by malignant lymphomas (13.9% ). In a similar series FORREST  reported tumour of the lacrimal gland as most common. Neurosurgeons,- DANDY,  VAN BUREN, PAPPEN AND HORAX -found meningiomas to be the commonest, comprising 31%. DRESCHER AND BENEDICT  also found meningiomas as the most common lesion of neoplastic, origin, which was followed by haemangiomas. PFEIFFER'S  observations were similar. O'BRIEN AND LEINFELDER  also observed that meningiomas were commonest but haemangiomas occupied the fourth place. BULLOCK AND REEVIS  also found meningiomas to be the most common cause of tumours causing unilateral proptosis. Therefore, to get the correct incidence of different orbital tumours one should study a series of consecutive cases attending the ophthalmic department and base the etiologic diagnosis on clinical evaluation, X-ray examination and surgical exploration with histopathological examination of the tissues removed.
In the present series, the commonest tumours happened to be lacrimal gland epithelial tumours (11 cases) and haemangiomas (10 cases). Next came reticulo-endothelial tumours (8 cases). It is interesting to note that there were 4 cases of carcinoma of unknown origin, whereas there was no case of meningioma in the whole series [Table 1] p. 161.
Benign primary orbital tumours were characterised by their slow growth and absence of inflammatory symptoms. Haemangioma was the most frequent type in this series. The tumour is generally believed to be congenital in origin but in 5 of our cases it appeared after the second decade of life. The average duration of proptosis in these cases was 4½ years. Haemangiomas of the orbit in adults are usually cavernous and encapsulated and are not often associated with other haemangiomas in the body (REESE  ), but we had a case where there were multiple haemangiomas all over the body.
Fibromata of the orbit are rare (DUKE-ELDER  ). They usually appear before the age of 16 and rarely after 30. The tumour in the present series appeared at an age which is unusual. Trauma has been mentioned in the etiology but no such history was available in the present case.
Primary sarcomas of the orbit are relatively common, Rhabdomyosarcomas are tumours of young age but of the 5 cases in the series, in 3 the tumour appeared quite late in life. Though they grow very rapidly, in 2 cases the progress was remarkably slow.
Reticulo-endothelial tumours of the orbit may appear as an isolated lesion or as a part of generalized disease. The prognosis is much better in the former group. Since ocular lymphatics are confined to the subconjunctival space and the lacrimal gland, most lymphomas of the orbit are located anteriorly. In the present series the lacrimal gland appeared to be the primary site in 3 instances. The eye lesion occured so insidiously in most of, the cases that the patients presented themselves not because of symptoms but because of proptosis. All lacrimal gland epithelial tumours are taken as potentially malignant, and therefore, the patient should be kept under observation for a long time. The typical clinical picture of a mixed tumour is an insidious onset of proptosis with the development of a palpable tumour in the upper-temporal quadrant of the orbit. The globe is usually displaced inwards, forwards and downwards. However, there were no clinical characteristics by which it could be differentiated from other expanding lesions occurring in the lacrimal gland fossa.
Neurofibroma is generally considered the commonest of, the neurogenic tumours of the orbit. The incidence in this analysis does not confirm this view. The tumour rarely occurs in the orbit without producing some evidence of the systemic disease (von Reclinghausen's neurofibromatosis) (Moss  ). In both the cases in this series the condition occured as an isolated lesion in the orbit.
Neurilemmomas have no characteristic clinical features except the fact that they are well encapsulated and occur in the orbit as an isolated lesion. However, it is to be noted that neurilemmoma may sometimes present as cases of retrobulbar neuritis with no evidence of the presence of a tumour, yet to miss the true diagnosis at this early stage can be disastrous. It is, therefore, imperative to exclude the presence of orbital neoplasm in all cases of retro-bulbar neuritis of obscure etiology.
Glioma of the optic nerve is a slow growing tumour; the proptosis is classically axial. However, in the late stages the displacement of the globe may be eccentric when the eye frequently deviates down and in REESE  ). Our observation is at variance with this. In 2 cases in this series the globe was displaced forwards, downwards and outwards and in one case the globe was displaced forwards, upwards and outwards. It is also interesting to note that the progress of the tumour was very rapid in one case due to a blunt trauma sustained just above the eye.
A series of 54 cases of primary orbital tumours causing unilateral proptosis were studied with regard to incidence and clinical features. Of them 51.8% were of mesenchymal origin, 27.8% of epithelial origin and 20.4%, were of neural origin. Lacrimal gland epithelial tumour was the commonest (20.4%) which was closely followed by haemangiomas (18.5%). Some of the rare lesions were pure fibroma, isolated neurofibroma and lymphoblastic lymphoma deep in the orbit. One case of neurilemmoma presented 3 years previously as a case of retrobulbar neuritis.
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