|Year : 1981 | Volume
| Issue : 1 | Page : 23-27
Tumour masquerading as pseudotumour of orbit
SP Dhir, KM Zafarullah, IS Jain, K Mohan, A Raina
Department of Ophthalmology and Pathology, Postgraduate Institute of Medical Education & Research, Chandigarh, India
S P Dhir
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dhir S P, Zafarullah K M, Jain I S, Mohan K, Raina A. Tumour masquerading as pseudotumour of orbit. Indian J Ophthalmol 1981;29:23-7
Pseudo-tumour of the orbit is a peculiar and a puzzling entity presenting problems in diagnosis and management. These are inflammatory lesions of the orbit of unknown aetiology and clinically resemble orbital tumours. Gogi and Nath in a consecutive study of 120 histologically proved cases of primary orbital tumours, described thirteen cases of non lymphoid pseudo-tumours. They are diagnosed clinically by exclusion of other aetiologies, by therapeutic response to systemic steroids and by histopathological examination of biopsy material. Nonetheless occasionally therapeutic response and histopathological examination of biopsy material are misleading.
We report 4 cases which were diagnosed as pseudotumour on the basis of histopathological examination and steroid response (3 cases, by clinicopath. examination and steroid response I case). These four patients on followup turned out to be true neoplasms.
| Case reports|| |
Case 1:- A 13 years female (CR No. A092466) presented with gradually progressive, painless proptosis of left eye of 15 days duration. Ocular examination revealed a firm, non- tender, non reducible swelling at the lateral 2/3rd of left upper orbital margin [Figure - 1] with limited elevation and abduction of eye ball. Left fundus showed papilloedema and retinal striae and there was reduction of visual acuity to 6/36. Systemic examination was normal except that the submandibular lymph nodes were enlarged, firm, non-tender, discrete and mobile. Clinical diagnosis of left lacrimal gland tumour was suggested. X-ray skull and PNS was normal. X-ray orbits showed soft tissue shadow in the left orbital region. Total leucocyte count was normal with marked small lymphocytosis (82%). ESR was slightly raised. No immature white cells were seen on repeated examination. Histopathological examination of the biopsy showed fibro-vascular stroma infiltrated by lymphomononuclear cells [Figure - 3]. She was diagnosed as a case of non specific, inflammatory pseudotumour of orbit. Patient was put on Inj.serepto-penicillin and Tab. prednisolone 60 mg per day. Proptosis regressed [Figure - 2] and vision improved to 6/6 and fundus became normal. After 2 months the patient presented to the medicine department with anaemia, generalised body aches and pains, splenomegaly and left cervical lymphadenopathy. Haematological examination revealed TLC 12,200/ cm. Differential leucocytic count was polymorphs 14%, lymphocytes 81%, Myelocytes 3% and blast cells2%. Bone marrow showed polymorphs 7%, lymphocytes 31% Myelocytes 45%. Metamyelocytes 3% and blast cells 14%. On the basis of systemic findings and haematological examination patient was diagnosed as a case of lymphoma breaking into the circulation.
Case II: GR, 5 years male (CR No. 554741) presented with gradually progressive proptosis of left eye of 20 days duration. On ocular examination, a firm irregular and nontender mass was palpable above and to the lateral side of the eyeball displacing it downwards and slightly outwards though the movements were equal on both sides.
Clinical diagnosis of pseudotumor of left orbit was put forth. Systemic, haematological and radiological examination revealed no abnormality. Patient was put on systemic antibiotics and steroids. Proptosis regressed. 3 months later patient again presented with proptosis of left eye and exposure keratitis [Figure - 4]. A biopsy of the orbital mass was obtained through anterior orbitotomy. Histopathological examination of the biopsy tissue revealed sarcoma, [Figure - 5] the further classification of which was not possible due to abundant degeneration and necrosis. Patient was put on radio-therapy. Proptosis became less.
Case III: RL, 33 years male (CR No. 032318) presented with redness and gradually progressive proptosis of right eye of 3 months duration which initially became less on systemic antibiotics and steroids but recurred after about one month.
On ocular examination, there was gross proptosis with chemosis of conjunctiva and restricted eyeball movements [Figure - 6]. In addition a firm non-tender mass was palpable in upper part of the orbit. Right fundus showed papilloedema. Systemic examination revealed non-tender hepatomegaly, no splenomegaly and no lymphadenopathy.
Clinical diagnosis of pseudotumour was made. All radiological and haematological investigations were within normal limits except polymorphonuclear leucocytosis with few atypical lymphocytes in the peripheral blood smear. Bone marrow examination was normal.
Biopsy showed mild fibrosis and mature lymphocytes [Figure - 7] and confirmed the diagnosis of pseudotumour. Patient was put on systemic steroids. Proptosis became less. Patient after 17 days presented to the medical department with pain in upper abdomen for last 4 days and some ocular complaints. He developed multiple nodules on the skin also. Revised diagnosis after considering the liver biopsy was Reticulum cell sarcoma.
Case : IV: K 21/2 years female (CR No. A 031315) presented with painful and gradually progressive proptosis of left eye of 2 months duration. There was history of injury 5-6 days prior to this.
On ocular examination, there was proptosis of left eye with conjunctival chemosis. The eye ball was displaced upwards and laterally with restricted movements in addition to a hard mass extending throughout the whole lower orbital margin [Figure - 8]. Systemic examination was normal.
Clinical diagnosis of rhabdomyosarcoma was made. X-ray chest and paranasal sinuses were normal. Haematological examination showed leucocytosis with slight lymphocytosis (58'.) and raised ESR (60 mm 1st hour). Bone marrow examination was normal. Histopathological examination of the biopsy material revealed fibrous tissue infiltrated by round to oval hyperchromatic cells. Some of the cells were lymphocytoid. She was diagnosed as a case of pseudolymphoma of orbit. Tumour responded poorly to systemic steroids and local radiotherapy to the orbit. After 6 months of treatment, patient died with systemic metastasis diagnosed as rhabdomyosarcoma at an other institution.
| Discussion|| |
The distinction between pseudo-tumours from true neoplasms of the orbit is of paramount importance. Pseudotumour of the orbit may be mistaken for a neoplasm thus causing unnecessary radical surgery. However, occasionally a converse problem arises as in the cases reported above when true neoplasms were mistaken for pseudo-tumours. Follow-up of 140 patients of pseudotumour by Blodi and Gass revealed that 8.4% of them developed such lethal diseases as Wagners's granulomatosis, midline granuloma, malignant lymphoma or leukaemia. In the present report three patients had sarcoma (reticulum cell, rhabdomyosarcoma and undifferentiated sarcoma) and one patient had lymphoma. Reese found 4 cases of Hodgekin's disease and one of the malignant lymphoma among his cases of non-specific chronic granulomas of the orbit. Gogi et al reviewing the clinicopathological features in fifteen cases of lymphoid pseudotumors stressed the general systemic examination, haematological investigation and lymph node biopsy to differentiate between lymphomas and benign lymphoid pseudotumour.
Retrospective histopathological review of the original orbital tissue was of no avail to reach a correct diagnosis. Only in the case No. 4 one could retrospectively entertain the possibility of an undifferentiated sarcoma.
Therapeutic trial with corticosteroids was very misleading and provided wrong assurance to the pathological diagnosis. It is possible that these tumours excite inflammatory reaction and thus some regression is seen with systemic corticosteroids. Case No. 1, who later turned out to be lymphoma, showed marked regression with systemic corticosteroids.
Since the pathologist's diagnosis of inflammatory pseudotumour and therapeutic response to corticosteroids does not eliminate the possible hidden lethal disease in the body, one should search thoroughly for a systemic disease and repeat biopsy should be carried out if the lesion progresses or recurs and these patients should be kept on close observation. An important clinical point which was overlooked in these case was that three of the patients were children and pseudo-tumours are uncommon in children. Thus despite histopathological report and clinical response to corticosteroids, the diagnosis of pseudo-tumours should be accepted with great caution.
| Summary|| |
Four cases of pseudotumors diagnosed by histopathology and favourable response to systemic corticosteroid are presented. Three of these patients on follow-up turned out to be sarcomas (reticulum cell, rhabdomyosarcoma and unclassified sarcoma) and fourth a case of lymphoma. Three of the four patients were under fifteen years of age. A thorough systemic examination, a close follow-up and repeat biopsy in case the lesion progresses or recurs, is suggested in cases of pseudo-tumours of the orbit. A diagnosis of pseudo-tumour in a child should be entertained with great caution.
| References|| |
Reese, A.B., 1976, Tumours of the Eye, Harper and Row Publishers, New York, pp. 538.
Gogi, R. and Nath, K.; 1977, Ind. J. Ophthalmol. 25 : 5.
Gogi, R., Nath. K. Khan, A.A. and Tyagi, S.P., 1976, Ind. J. Ophthalmol. 24: 26.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]