|Year : 1989 | Volume
| Issue : 4 | Page : 191-192
Atypical lymphoid hyperplasia (lymphoid tumour of indeterminate nature) of orbit-A case report
Ajoy Paul, AK Deb, S Chakravarty, SR Das
Professor Of Ophthalmology, Silchar Medical College, Silchar - 788014, ASSAM, India
S R Das
Professor Of Ophthalmology, Silchar Medical College, Silchar - 788014, ASSAM
Atypical lymphoid hyperplasia, a rare variety of orbital tumour occurring in a 60 year old male patient is presented with review of literature with special relation to its diagnostic and prognostic difficulties.
|How to cite this article:|
Paul A, Deb A K, Chakravarty S, Das S R. Atypical lymphoid hyperplasia (lymphoid tumour of indeterminate nature) of orbit-A case report. Indian J Ophthalmol 1989;37:191-2
|How to cite this URL:|
Paul A, Deb A K, Chakravarty S, Das S R. Atypical lymphoid hyperplasia (lymphoid tumour of indeterminate nature) of orbit-A case report. Indian J Ophthalmol [serial online] 1989 [cited 2014 Mar 17];37:191-2. Available from: http://www.ijo.in/text.asp?1989/37/4/191/26050
| Introduction|| |
Orbital lymphoid tumour is an example of extranoda lymphoid tumour.  Often these lesions pose confusior and controversy regarding their diagnosis and biological behaviour .  Reactive lymphoid hyperplasia,Atypica lymphoid Hyperplasia and Malignant lymphomas are common types of orbital lymphoid lesions. Amonc these, atypical lymphoid hyperplasia group poses great est problem in diagnosis on the basis of histological anc cytological examination. It is also difficult to predict the prognosis of these group of lymphoid tumours. Atypica lymphoid hyperplasia is a rare lymphoid neoplasm it the series of Knowles et al. 
Because of rarity and formidable clinical and pathological problems encountered in diagnosing and predicting the prognosis of these lesions, a case of `Orbital Atypical Lymphoid Hyperplasia' is presented here.
| Case report|| |
MMD, a 60 year old Hindu Male reported to us or 15.3.88 with a painless swelling in the right lower eyelic and mild proptosis for three years. Local examination; revealed a 3 cm by 2 cm size, firm, elliptical swelling it medical side of right lower eyelid, with engorged super ficial veins, mobile from side to side but relatively fixer from above downwards. Proptosis was 4 mm eccentric with slight upwards and outwards deviation of the globe Ocular motility was unrestricted, vision, fundoscopy and intraocular tension was normal. Systemic exami nation of lymphoreticular system, respiratory system cardiovascular system, gastrointestinal system shower no abnormality. [Figure - 1]
Routine blood examination, urine analysis, orbital skia gram, orbital venography, revealed no abnormality The tumour was removed en mass by anterior orbito tomy approach. Post operative period was uneventful and the patient is under close follow up now.
Microscopic examination of the tumour showed a dill fuse lymphoid cell population composing of two distinct types. One group of cells were mature lymphocytes while other lymphoid cells showed borderline cytological immaturity. Adjacent muscle fibre and fatty tissue
| Discussion|| |
Few subjects have created more controversies than orbital lymphoid tumours. Normally there is no lymphoid tissue in the orbit and so any lymphoid mass in the orbit is considered to be abnormal. Orbital lymphoid lesions constitute about 10 % of all orbital tumours . 
Orbital lymphoid neoplasms have been classified into
(a) Inflammatory Pseudotumour.
(b) Benign Reactive Lymphoid Hyperplasia.
(c) Atypical Lymphoid Hyperplasia.
(e) Malignant Lymphoma . 
Malignant lymphoma constitute 50 % of all orbital lymphoid tumours, the remaining 50 % include benign reactive lymphoid hyperplasia and Atypical Lymphoid Hyperplasia. 
Usually pathologists do not face much problem in diagnosing benign lymphoid hyperplasia or a frankly malignant lymphoid tumour of the orbit. But atypical lymphoid hyperplasia cannot be straightway categorized either a benign lesion or a malignant one because these tumours share same properties of benign lymphoid hyperplasia as well as cell population shows same degree of immaturity.
Atypical lymphoid hyperplasia is a diffuse proliferation of lymphoid cells where the cells show borderline maturity or may show a subpopulation of abnormal cells with large hyperchromatic nuclei along with mature lymphocytes. Like benign lymphoid hyperplasia they do not show (a) characteristic polymorphic cell population consisting of lymphocytes, plasma cells psinophili8 and histiocytes, (b) typical germinal follicles and mitotic figures. In the same way the unequivocal malignant cells of malignant lymphomas are not present in Atypical lymphoid hyperplasia. Another feature of Atypical lymphoid hyperplasia is its tendency to invade the adjacent muscle and fat and in the present case also, the tumour was found to have infiltrated inferior rectus muscle.
Orbital lymphoid lesions pose another formidable difficulty in predicting their clinical behaviour. Even if accurate histopathological diagnosis is made, in many cases it is not possible to predict their future course accurately. For example, 15 % to 25 % histopathologically confirmed cases of benign lymphoid hyperplasia and 40% of atypical lymphoid hyperplasia show concomittent or subsequent systemic lymphoid tumour.  In the present case the patient has lesion confined to his orbit for last three years without any systemic manifestation now and he has been kept under close follow up. The 5 year mortality rate in case of atypical lymphoid hyperplasia is 19 %, whereas it is 6 % benign lymphoid hyperplasia and 58 % in malignant lymphomas. 
Because of doubtful prognosis, all orbital lymphoid neoplasms are thoroughly investigated and followed up at regular intervals. All effort should be made by using all relavant diagnostic procedures to exclude the presence of any ocultlymphoid tumour in any other organs or tissues. If the lesion is found to be confined exclusively in the orbit, then local irradiation is the treatment of choice irrespective of histopathological type . ,,, In atypical lymphoid hyperplasia usually a radiation dose of 1500 to 2000 rads are given with adequate protective measures to protect the cornea.  Surgical removal is not satisfactory because of the infiltrative nature of the lesion. After local radiotherapy periodic follow up of the case at the interval of 6 months fog 2 years is mandatory.
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[Figure - 1], [Figure - 2]