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Year : 1971  |  Volume : 19  |  Issue : 2  |  Page : 61-66

Primary reticulo-endothelial tumours of orbit

Department of Ophthalmology and Pathology, Irwin Hospital and Maulana Azad Medical College, New Delhi, India

Correspondence Address:
Hari Mohan
Department of Ophthalmology and Pathology, Irwin Hospital and Maulana Azad Medical College, New Delhi
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Source of Support: None, Conflict of Interest: None

PMID: 15744971

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How to cite this article:
Mohan H, Sen DK, Chatterjee P K. Primary reticulo-endothelial tumours of orbit. Indian J Ophthalmol 1971;19:61-6

How to cite this URL:
Mohan H, Sen DK, Chatterjee P K. Primary reticulo-endothelial tumours of orbit. Indian J Ophthalmol [serial online] 1971 [cited 2024 Mar 2];19:61-6. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1971/19/2/61/34990

Table 1

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Table 1

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The orbit may be affected in reticulo-endothelial tumours. This usually occurs late in the disease and there is no great problem in the differential diagnosis; biopsy is rarely indicated. Occasionally, however, orbit is the first and the only part of the body involved (O'Brien and Leinfelder[7], de Treigry[12], Reese[8], Tavares[11], van Wien [13] , Forrests) and in that event diagnosis as to the nature of the tumour is extremely difficult without biopsy as systemic exa­mination and blood picture are usually of little help in all such cases.

We came across fourteen cases of reticulo-endothelial tum­ours in our special clinic over a period of 10 years. In 6 of them, orbital involvement was late during the course of an al­ready generalized lymphomatous disease. In 3 cases proptosis was the presenting feature of the dis­ease but by the time patients re­ported to the hospital the condi­tion had progressed to involve the other parts of the body. Only in the remaining five cases the af­fection was clinically limited to the orbit when first seen.

The purpose of this communica­tion is to present the five cases in the last category along with the results obtained with radiothe­rapy.

  Case Reports Top

Case 1, a 49-year-old male, pre­sented with protrusion of the right globe and a gradually in­creasing swelling in the upper and inner quadrant of the orbit for about 3 years. The right globe was displaced downwards and for­wards [Figure - 1]. On palpation through the upper lid the mass was soft and appeared to extend forwards from deep in the orbit. Overlying skin was normal and free from the swelling. Exoph­thalmometric reading (Hertel) 26 mm. (normal left eye 15 mm.). Elevation of the right eye was de­fective. Visual acuity 6/12; (nor mal left eye 6/6). Fundus exami­nation revealed papilloedema.

Systemic examination disclosed no abnormality. Skiagraph re­vealed enlargement of optic fora­men on the right side. Blood picture and bone marrow study were normal. Rest of the invest­igations yielded negative informa­tion. Orbit was opened up by Kronlein's procedure and major part of the growth was removed [Figure - 2]. Histopathological exami­nation revealed the tumour mass composed of uniform round cells with scanty basophilic cytoplasm and large nuclei many of which contained fairly prominent nuc­leoli. Mitotic figures were seen in moderate numbers-a picture compatible with lymphoblastic lymphoma [Figure - 3].

As the whole of the tumour could not be removed the patient was put on radiotherapy. With a total dose of 1500r spread over a period of 2 weeks the proptosis disappeared rapidly and complete­ly. Visual acuity improved to 6/9. There was no recurrence during a follow-up period of 6 years.

Case 2, a 12-year-old female, presented with one year's history of steadily increasing proptosis on the right side. The globe was almost dislocated [Figure - 4]. Palpa­tion through the upper lid reveal­ed a firm mass in the orbit which was not tender. There was no systemic involvement. Repeated blood and bone marrow studies were normal. Other investigations were all negative. The eye was enucleated as it was blind. Biopsy of retrobulbar mass proved it to be a lymphoblastic lymphoma.

She received a total dose of 2000r in 5 applications. Response was very good. No recurrence after a follow-up period of 5 years.

Case 3, a 25-year-old female reported to the hospital with 2 years, history of right proptosis and irregular swelling in the upper and upper--inner part of the orbit extending on to the nasal region and forehead. There was history of operation done else­where about 4 weeks before she reported to the hospital which re­sulted in a non-healing irregular wound at the medical part of the swelling. The right globe was grossly deviated forwards, down­wards and outwards [Figure - 5]. Conjunctival and retinal vessels were engorged but no papilloe­dema. Exophthalmometric read­ing (Hertel) 28 mm. (normal left eye 14 mm.). Visual acuity 6/18 (normal left eye 6/5). Palpation revealed a firm irregular mass which was not tender. There was no systemic involvement. All in­vestigations including repeated differential blood count and study of bone marrow were negative ex­cepting skigraphy which showed some reactive hyperplasia of the supra-orbital margin. Anterior orbitotcmy was done to remove a piece of tissue. Histopathological examination ' revealed this to be again a case of lymphoblastic lymphoma. She was put on radio­therapy. A decided improvement was noticed immediately after the first exposure and proptosis disap­peared almost completely after the completion of therapy. The eyesight improved to 6/9. The patient was last seen after a period of 5½ years. No recurrence of the tumour was in evidence. How­ever, mild degree of residual pro­ptosis was noticeable.

Case 4, a 35-year-old female, reported to the hospital with the history of gradually increasing protrusion of the left globe with deterioration of vision and bulging of left upper lid for about 1 year.

She had been hit on the forehead by a stone just above the left eye 6 months previously. On palpa­tion through the upper lid a soft to firm irregular mass was felt in the orbit. Superior orbital margin was irregular and tender on pre­ssure. Left eye ball was displaced forwards and downwards [Figure - 6].

Vision 6/9. Systemic examination revealed no abnormality. Blood picture and bone marrow studies were normal. Other investigations gave negative results. Skiagraph revealed thickening of the supra orbital margin. Biopsy revealed the tumour to be reticulum cell sarcoma. Histopathological section showed uniform spreading of the tumour cells having abundant cytoplasm and vesicular nuclei with distinct nucleoli. Many of the nuclei were infolded giving them a reniform appearance [Figure - 7].

She was put on radiotherapy. With a total dose of 2500r over a period of 3 weeks proptosis dis­appeared almost completely. Fol­low-up for a period of 7 years re­vealed no recurrence.

Case 5, a 24-year old female reported to the hospital with Hodgkin's disease which manifes­ted initially in the left orbit. The diagnosis was established by biopsy. Response to radiotherapy was quite satisfactory. No recur­rence after a period of seven years (Sen, Mohan and Chatterjee [10] ).

  Discussion and Comments Top

Tumours arising from reticulo­endothelial system are classified according to their tissue of origin. Lymphomas are neoplasia of the lymphatic line whereas reticulnm cell sarcoma is a neoplasia of the reticular line. Hodgkin's disease involves both lymphocytic and re­ticular lines. Any of these tum­ours may involve the orbit primarily or as a part of genera­lized involvement. Lymphoid tissue around the eye is most commonly encountered sub-con­junctivally and in the lacrimal gland. It is at these sites, there­fore, the lymphomatous tumours are most likely to appear. Such tumours deep in the orbit are usually in the nature of reticulum cell sarcoma (Reese[9]). It is of great interest to note ,hat we came across 3 cases of lympho­blastic lyphoma deep in the orbit where there is normally no lym­phoid tissue. Such occurrence is ascribed to the presence of foetal remnants and pre-existing lym­phoid elements (Coats [1] ).

Reticulo-endothelial tumours of the orbit have been regarded as uncommon (Duke-Elder [2] ). Recent­ly a 10 to 20 percent. Incidence has been reported (Reese, [9] Mortada[5], Haye and Haut[4]). In our series the incidence has been 8.5 per cent. of all proptosis cases and 17.1; per cent. of all primary orbital tumours. The lymphoblas­tic lymphomas are highly diffe­rentiated tumours and of benign clinical habit; Lymphadenoma (Hodgkin's disease) may also be­have in a similar fashion (case 5); but it is very rare for a reticulum cell sarcoma to remain confined to the orbit for any length of time. These lesions may arise at any age. The onset is so insidious that the patients present themsel­ves not because of symptoms but because of cosmetic disfigurement either in the form of a mass under the conjunctiva or skin or dis­placement of the globe. Diagno­sis is usually established by bio­psy after excluding the other possible causes of proptosis. His­tological typing of such tumours is very important from the point of view of prognosis as to life ex­pectancy but may at times be ex­tremely difficult because of their common origin. The picture is so variable that some of the cases are even misdiagnosed as chronic granulomas especially in the early stages. The cases we came across, however, were typical and did not present much difficulty in establishing the nature of the lesion. Radiological evidence of enlarged optic foramen in some cases (case 1) may be misleading. Difficulties are also encountered with while removing them surgi­cally as they are not encapsulated. Fortunately they are all highly radiosensitive. Radiotherapy at a stage when the lesion is clinically confined to the orbit have been most encouraging. In three cases there was complete regression of proptosis and in the remaining two cases regression was considerable. A 5-7 years' follow-up revealed no recurrence in any of the 5 cases. Prognosis, therefore, ap­pears to be very good with radio­therapy in patients with lesions clinically confined to the orbit.

  Summary Top

The incidence of reticulo-endo­thelial tumours has been 17.1 per cent. of primary orbital turn­ours. Only in 5 cases the lesion was clinically confined to the orbit when first seen. The results of irradiation in these type of lesions have been most encouraging. A 5-7 years' follow-up shows per­manent regression of the tumour in all the 5 cases.

  References Top

COATS.: Arch. Ophthal. 44. 221, 235. (1915). Cited in 2 .  Back to cited text no. 1
DUKE-ELDER. S.: Text Book of Ophthalmology. Vol. V, PP. 5348­-5555. Henry Kimptoo, London. (1952.)  Back to cited text no. 2
FORREST, A. W.: Intraorbital Tumours, Arch. Ophthal (Chicago), 41, 198, (1949).  Back to cited text no. 3
HAYE, C AND HAUT, J.: Arch. Ophthal. (Paris). 26, 133 (1966). Cited in 6.  Back to cited text no. 4
MORTADA, A.: Nature of lym­phoid Tumours of the Orbit, Con­junctive, Eye lids and Lacrimal Gland. Amer. J. Ophth. 57, 320, (1964).  Back to cited text no. 5
NOLAN, J.: Reticulo-endothelial Tumours of the Orbit. Brit. J. Ophth. 52, 532. (1968).  Back to cited text no. 6
O'BRIEN, C. S. AND LEIi1'ir EL­DER, P. J.: Unilateral Exopi- thal­mos. Amer, J. Ophth. 18, 123, (1935).  Back to cited text no. 7
REESE, A. B.: Orbital Tumours and their Surgical Management. Amer. J. Ophth. 24, 386, 497, (1941).  Back to cited text no. 8
REESE, A. B.: Tumours of the Eye, 2nd Ed., pp. 533 et seq. Hoeber, New York. (1963)  Back to cited text no. 9
SEN, D. K. MOHAN, H. AND CHATTERJEE, P. K.: Hodgkin's Disease of the Orbit. (In Press).  Back to cited text no. 10
TAVARES.: Bol. S. Portug., Oft., V. 61. (1947). Cited in 2.  Back to cited text no. 11
DE TREIGNY.: Bull.s, d'o Paris, 48, 406, (1936). Cited in 2.  Back to cited text no. 12
WIEN, S. VAN.: Lymphocytoma of the Orbit successfully treated by Roentgen Irradiation. Amer, J. Ophth. 31, 209. (1948).  Back to cited text no. 13


  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7]

  [Table - 1]


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