Vol.13
No. 1, October 2003
DIAGNOSIS AND MANAGEMENT OF ORBITAL LYMPHOID TUMOURS
Dr.
Usha Singh, Dr. Sristhi Raj, Dr. Moheep Singh,
Dr. Supratik Bandyopadhyay.
INTRODUCTION
Lymphomas
generally occur in lymphnodes, but may occasionally develop in
extranodal sites such as the orbit. Among different
causes genetic alterations in the chromosomes, chemical agents
( herbicides / hair dyes), viral infection (EB virus) are
thought to be responsible.1-2 Non-Hodgkins
lymphoma (NHL) present more commonly in the orbit than
Hodgkins Lymphoma and comprises from 4 to 24% of all orbital
masses.3 They are highly radiosensitive and
local recurrence is unusual.4-5 In the recent
past development of new reagents (monoclonal antibodies, DNA
& RNA probes), techniques ( tissue section
immunohistochemistry, immunofluorescence, immunoblotting, PCR)
and new machines ( cytocentrifuge, epifluorescence
microscopes, flow cytometers and automated immunostainers) has
thrown new light in the diagnosis and management of lymphomas.
The aim of the present study was to find out various clinical
presentations and outcome of treatment of orbital lymphoid
tumours.
MATERIAL
AND METHODS
This
was a retrospective study in which data of the patients who
had histologically proven lymphoid tumours of the orbit and
presented at our oculoplasty clinic between January 1996 to
August 2002 were analyzed. A detailed history and
complete ocular and systemic examination, peripheral blood
counts, serum chemistry panels ( RFT, LFT) radiographic
studies (CT liver, spleen, retroperitoneal area, bones, brain
and other suspected sites) with or without bone marrow biopsy
was done to determine the nature of disease. If the
tumour was resectable, complete removal of the mass was done
otherwise incision biopsy was performed and enough tissue was
retrieved to perform immunohistochemical and cell marker
studies. Ann Arbour system and "REAL" SYSTEM
of classification was used.
RESULTS
A
total of 13 cases were seen. There were 9 male and 4
female patients. Age ranged from 5 to 90 years ( Mean
51.50). Follow-up ranged from 1-6 yrs. ( mean 1.4 yrs).
Twelve patients had unilateral disease and one had bilateral
disease. Interestingly all 13 patients had primary
orbital disease without any systemic involvement. Among
different clinical presentations proptosis was the most
common finding, present in 7 out of 13 patients followed by
lid swelling and palpable mass lesion in 5 patients,
subconjunctival mass lesion in 2 and myositis in 2 patients.
Nine out of 13 patients had USG of the orbit done which showed
hypoechoic mass lesion. One patient was initially
diagnosed as reactive lymphoid hyperplasia on histology but
later progressed to bilateral NHL. CT scan was done in all of
the 13 patients which showed extraconal (10), intraconal(2),
intraconal & extraconal (1) mass lesion present in the
orbit. Histopathology showed low grade lymphoma in 5,
high grade in 1 and intermediate grade in 1 patient.
Histochemical staining was performed in 2 patients which
showed positive staining with CD 20. Three out of 13 patients
received radiotherapy, 3 patients were advised chemotherapy
whereas 4 patients received combined radiotherapy and
chemotherapy. One patients who recently came to us had
an excision biopsy done and has been referred for
radiotherapy. Two out of 13 patients were lost to follow-up
after they were referred for radiotherapy. Complications
of radiotherapy was seen in 4 patients, 1 had decrease in
vision and 1 had persistent epithelial defect and required
tarsorraphy and 2 patients developed dry eyes. All 10
patients who received treatment and came for regular
follow-up were disease free at last follow up.
CONCLUSION
Primary
lymphoid tumours of the orbit are not very uncommon.
Although proptosis is the most common presentation patient may
also present with lid swelling. Histopathology remains
the goal standard for diagnosis. Timely diagnosis and
prompt management can result in excellent prognosis.
Radiotherapy is the treatment of choice for primary orbital
lymphomas and recurrences are unusual.
REFERENCES
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Ness
GO et al. Invest Ophthalmol Vis Sci 2002; 4(1):
9-14.
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White
WL et al. Ophthalmology 1995 ; 102 (12): 1994-2006.
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Demirci
H, Shield JA et al. Ophthalmology 2002 ; 109 (2): 24 -8.
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Esmaeli
B et al. Ophthal Plast Reconstr Surg 2002 ; 18 (4):
247-53.
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Erkal
HS et al. Tumor 1997; 83 (5) : 822-825.
Department
of Ophthalmology,
Post Graduate Institute of Medical Education and
Research, Chandigarh
