|Year : 1976 | Volume
| Issue : 1 | Page : 26-31
Lymphoid pseudotumours or pseudolymphomas of the orbit
R Gogi, K Nath, AA Khan, SP Tyagi
M.U. Institute of Ophthalmology, Aligarh, India
M.U. Institute of Ophthalmology, Aligarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gogi R, Nath K, Khan A A, Tyagi S P. Lymphoid pseudotumours or pseudolymphomas of the orbit. Indian J Ophthalmol 1976;24:26-31
A pseudotumour is an inflammatory lesion of orbital tissue of unknown etiology, clinically simulating an tumour. The term pseudotumour is misleading, as the condition presents quite often as a true tumour though it is an inflammatory mass and not a neoplastic one. Alternatively the term "nonspecific granuloma" has been used. Since the granuloma is characterised by the presence of epithelioid cells and giant cells and such a histological picture is not always present, therefore, this term is also not correct. Hence, in the absence of an appropriate name and exact etiology of this clinical entity the term pseudotumour remains in use.
Hartung in 1845 reported the first case of orbital pseudotumour in a suspected case of malignancy. Host ever, the term pseudotumour was introduced by Birch-Hirschfeld in 1905.
Birch-Hirsclhfeld reviewed thirty cases of orbital pseudotumours from the literature. He included some of his own cases and divided them into the following three groups;
(a) Tumours which subsided spontaneously or following medical treatment with potassium iodide, mercury, arsenic or quinine.
(b) Cases of proptosis in which no tumour was found on operation and microscopic examination revealed chronic inflammatory tissue.
(c) Cases of proptosis in which an orbital mass was detected, removed and the histological examination revealed a chronic nonspecific inflammatory mass containing diffuse follicular areas.
Thereafter, pseudotumours had been classified in different ways by Dunnington and Berke, Hogan and Zimmerman, Reese, and Agarwal et al. However, it was the classical description of Hogan and Zimmerman and Zimmerman, which gave the pseudotumours its present status.
| Material and Methods|| |
In the present report clinico-pathological features of fifteen cases of lymphoid pseudotumours from a study of one hundred and twenty primary orbital tumours are described. Each case was thoroughly examined and fully investigated. Only histo-pathologically proved cases are included in this article. Macro- and microscopic appearances of these new growths were studied in each case.
| Observations|| |
Amongst the fifteen cases, six cases belonged to second decade, three cases each to third and fourth, one to fifth and two cases to sixth decade of life. There were eleven male and four female cases. Apart from protrusion of the eyeball in all the patients, there was history of pain in the orbit (8 cases), gradual diminution of vision (7 cases), watering (5 cases) and ptosis (3 cases). All the cases were unilateral and the duration of proptosis varied from three months to two and a half years.
On examination, proptosis was found to be slightly eccentric in eleven cases [Figure - 1],[Figure - 2] and axial in the other four [Figure - 3]. The maximum amount of proptosis was upto 20.0 mm. in one case. All cases manifested increased intraorbital pressure and varying amount of limitation of ocular movements. The tumour mass was palpable in twelve cases. Other features such as diminution of vision (7 cases), defective pupillary reaction (6 cases), lid oedema and chemosis (3 cases), ptosis (3 cases), fund us changes in the form of early papilloedema and corresponding visual field defects were also noted in two cases. On radiological examination there was increased soft tissue shadow in the orbital region in all the cases. Orhitovenogram showed displacement of the first and second parts of superior ophthalmic vein in six cases when the tumour either occupied the upper inner quadrant or roof of the orbit [Figure - 4].
General systemic examination and laboratory tests were all normal.
The tumour mass was removed in all the cases either by lateral (11 cases) or anterior (1 case), or transfrontal orbitotomy (I case) and in two cases of pseudotumours excision of pseudotumours alongwith enucleation of the eyeball was done. The tumours occupied floor of the orbit (7 cases), upper medial quadrant (3 cases), roof of the orbit (3 cases) and filled whole of the orbit behind the eyeball in two cases
[Figure - 5]. Often the tumours which insinuated in between the orbital tissues were removed in pieces.
(a) Gross appearance : It varied greatly. None of the lymphoid pseudotumours was encapsulated. They were well circumscribed with nodular surface in eight cases [Figure - 6], in another six the mass was irregular without any specific shape [Figure - 7]. In one case there was a small stone impacted in the pseudotumour [Figure - 8]. It was roughly biconvave and measured 6 x 4 x 4 mm [Figure - 9]. On chemical analysis, the stone consisted of calcium, phosphate and carbonate.
Consistency of the tumour mass varied from soft to firm and colour from grey to creamy white or greyish white. Cut surface was homogenous in all except two cases where the appearance was granular.
(b) Microscopic appearance : All the cases were characterized by the presence of lymphoid follicle formation with fibrous tissue proliferation in between [Figure - 10]. In the follicles there were pale and deeply staining areas [Figure - 11]. Dark areas consisted mainly of lymphocytes and pale areas contained a large number of reticulum cells [Figure - 12]. There was no evidence of malignancy. In one case there was no follicle
formation but there was diffuse proliferation of lymphocytes, reticulum cells, polymorph, eosinophils and plasma cells without any evidence of malignancy. General systemic examination and haematological investigations did not show anything positive in these cases.
| Discussion|| |
More than three-fourths of the cases (12 cases) in the present report have occurred in the second, third and fourth decades. Silva also found these cases to be more frequent under thirty years of age. On the contrary Blodi and Gass reported this to be more during fourth, fifth and sixth decades. Coop, and Blodi & Gass observed that the cases are almost evenly distributed in both the sexes, whereas Ingalls, and Silva reported that pseudotumours occur almost twice in females. In the present series, males are more commonly affected.
Lid oedema and chemosis was noticed only in three cases whereas diminution of vision and defective pupillary reaction were common findings along with the proptosis. Ingalls, Coop and Blodi & Gass reported, that oedema of lids and conjunctiva are common clinical findings. The low incidence of changes in the lids and conjunctiva may be due to the fact that our cases reported very late and by that time orbital circulation get adjusted to the new environment. Similarly visual changes are due to deviation of the eyeball with resultant amblyopia and pupillary changes and as a result of direct pressure by the tumour mass on the nerve fibres.
To distinguish a benign from malignant lymphoid lesion elsewhere in the body, the integrity of the architecture of lymph node is an important criterion. In the orbit, however, lymph nodes are not present and histological distinctions between lymphocytic infiltrations from benign and malignant lymphatic tumours is often difficult. Perhaps this is the reason that in the past such lesions have been diagnosed as cases of lymphomas or lymphosarcomas by Forrest, Das and Mohan et at Hogan and Zimmerman pointed out that any such lesion of the orbit without any general systemic manifestation and with normal haematological investigations, should be labelled as "Lymphoid Pseudotumour". A similar lesion takes place in the lungs and is labelled as "Pseudolymphoma". Liebow et al suggested that the absence of a widespread disease is the best criterion for distinguishing pseudolymphoma from lymphomas. Further, the former is largely composed of mature lymphocytes and regional lymph nodes are not involved. We feel that this approach of lung pathology can be well adopted for orbital pseudolymphomas, i.e., lymphoid pseudotumours of the orbit.
| Summary|| |
In a study of 120 cases of primary orbital tumours, fifteen cases of lymphoid pseudotumours have been reported. Clinical and pathological features of these lesions have been described in detail. The term "Pseudolymphomas" in place of lymphoid pseudotumours of the orbit is suggested.
| References|| |
Agrawal, P. K., Dayal, Y., and Agarwal, L. P. 1967, Orient. Arch. Ophthal., 5, 263.
Birch-Hirschfeld, A. 1905, Ber. Dtsch. Ophthal.
Ges., 32, 127.
IBID 1915 Graefe-Saemisch Hb. D. ges. Augenheilk, 2nd Ed., Leipzig. 9 (1). P. 556.
IBID 1930, Quoted Coop, M.E. (1961).
Blodi, F.C., and Gass, J.D.M. 1967, Trans. Amer. Acad. Ophthal. Otolaryng., 71, 303.
Coop, M.E. 1961; Brit. J. Ophthal., 45,
Das, S.P. 1963; J. All India Ophthal. Soc.,
Dunnington, J.H. and Berke, R.N. 1943; Arch. Ophthal.,
Easton, J.A., and Smith, W.T. 1961, Jour. Path. Bact„
Forrest, A.W. 1949, A.M.A. Arch. Ophthal.,
Hartung, 1845; Ann. Oculist, Paris, 14, 164.
Hogan, M.J., and Zimmerman, L.E. 1962; "Ophthalmic Pathology-An Atlas and Text Book", P. 763, W.B. Saunders Company, Philadelphia and London.
Ingalls, R.C. 1953, "Tumours of the Orbit and Allied Pseudotumours". Thomas, Springfield, iii, Blackwell Scientific Publications, Oxford.
Liebow, A.A., Carrington, C.R.B., and Friedman, P.J. 1972; Hum. Path., 3,457,
Mohan, H., Sen, D.K., and Gupta, D.K. 1968, Orient. Arch. Ophthal., 6,
Reese, A.B. 1963, "Tumours of the Eye", Ed. 2nd, P. 538, Hoeber, N.Y.
Silva, D. 1968, Amer. J. Ophthal., 65,
Zimmerman, L.E. 1964, Lymphoid tumours. In ocular and adnexal tumours: New and controversial Aspects. pp. 429-446. Kimpton, London, Mosby, St. Louis.
Zimmerman, L.E. 1967, Arch. Ophthal.,
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11], [Figure - 12]