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Malignant Tumour of Lacrimal Gland
HL Trivedi*, S Murade**, K Kamath***, AB Ingole***, C Sahu**, S Chavan**
 
Abstract
Adenocarcinoma of lacrimal gland is rare in a child of 12 years and its rapid progress to such a large size in such a short time is unusual. Proptosis of such a severe degree is also unusual.
 

Introduction
Adenocarcinoma of lacrimal gland is a rare tumour with high morbidity and mortality. Usually lacrimal gland tumour occurs after age of 35 years and only 8.6% are malignant. They commonly present during fourth to sixth decades of life. We report an unusual case of adenocarcinoma of lacrimal gland in 12 year old child.

Case Report
A 12 year old male child presented with complain of painful swelling of right eye upper lid with impaired vision since 2 months. On examination visual acuity in right eye was no perception of light and in left was 6/6. There was swelling of lid with chemosis of conjunctiva with conjunctival and ciliary congestion. An external examination of right eye showed swelling with fullness in superotemporal region. There was proptosis with inferomedial displacement measuring 35 mm. The cornea showed exposure keratitis and details of anterior chamber, iris and posterior segment were not visible due to it. The left eye had 6/6 vision, with normal external and posterior segment. Extra-ocular movements of right eye were markedly reduced in all direction. Extra-ocular movements of left eye were normal. C.T.Scan of orbit showed local invasion of orbital tissue and skin. On systemic examination there was no abnormality detected. There were no distant metastasis. Haemoglobin and peripheral blood smear were normal, E.S.R. was 87 mm. in1st hour. X-ray chest was normal. Systemic examination showed no abnormality. There was no lymphadenopathy. F.N.A.C. showed features of adenocarcinoma. In view of a huge, potentially malignant tumour, and nonseeing eye an exenteration was considered appropriate in our patient.



Fig. 1 : Lateral view.


Fig. 2 : Oblique view.

Discussion
Tumours of lacrimal gland are relatively rare, consisting 5 to 7% of space occupying lesions of orbit. Amongst them 58.6% are mixed tumours and most of them are benign. Only 8.6% are malignant. They usually occur in adults, commonest age group is 40 to 50 years. Malignant tumours are seen after age of 70 years and very rarely in children. The tumour appears as palpable mass in the upper and outer quadrant of the orbit, hard nodular and slightly mobile lying just underneath the orbital rim. Proptosis with infero-medial displacement of eye with limitation of ocular movement particularly upward and outward. Lid oedema, transient diplopia and loss of vision may occur. Due to lack of pain most of the cases have clinical signs for 1 to 4 years. Almost 50% of tumours are pleomorphic adenoma.(Mixed cell tumour). It has two distinct elements, one is epithelial lining of the ducts and other has an apparently mesenchymal appearance. The stroma is formed by outer layer of epithelium that lines the ducts. Metaplasia of epithelium forms myxoid, fibrous and cartilaginous stroma. Compression of surrounding tissues forms psedocapsule, with infiltration through it. X-ray of orbit shows enlargement of lacrimal gland fossa, without bone destruction. Treatment is by total excision, if, incomplete; it can recur with tendency to malignancy. Lateral orbitotomy is done excising entire tumour with pseudocapsule surrounding levator aponeurosis conjunctiva and periorbita must be excised en bloc to avoid recurrence and misery to patient.

Among the malignant tumour 60% are adenocystic carcinoma. 20% are mixed cell tumour and 20% are undifferentiated. Adenocystic carcinoma (old name cylindroma) on microscopic examination shows closely packed small densely stained cells aggregated around large ovoid spaces which contain hyaline or mucin.Under low power of microscope Swiss cheese appearance is seen. Five patterns are seen, cribriform, sclerosing, basaloid, comadocarcinoma,and tubular. Prognosis is worst with basaloid. 5 year survival is only 21%. If there is no basaloid component 5 year survival is only 71%. Majority of death occur due to intracranial extension by perineural extension. Lung metastasis is usually seen 5 to 10 years after diagnosis. Secondary tumour may rarely occur due to extension from P.N.S. or squamous cell carcinoma of conjunctiva, basal cell carcinoma of skin, sebaceous carcinoma of lid Lymphoproliferative disease involving lacrimal fossa include lymphosarcoma and Burkit’s lymphoma, which is commonest cause of bilateral lacrimal gland involvement in Uganda. Hodgekin’s disease may rarely involve both lacrimal glands. Radiotherapy and chemotherapy may be tried and may be effective in some patients.

References

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*Assoc. Prof.; **Resident; ***Lecturer, Dept. of Ophthalmology, TNMC and BYL Nair Ch. Hospital, Mumbai Central, Mumbai 400 008.

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