|Year : 1987 | Volume
| Issue : 1 | Page : 44-48
Neurofibrosarcoma of lacrimal gland
SP Pattanayak, JS Mathur, V Thakur, S Khanna
Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005, India
S P Pattanayak
Institute of Medical Sciences, Banaras Hindu University, Varanasi - 221 005
| Abstract|| |
A rare case of neurofibroma of lacrimal gland showing malignant transformation in a male patient eye 31 years is reported. There was no evidence of recurrence 'on a follow up study.
|How to cite this article:|
Pattanayak S P, Mathur J S, Thakur V, Khanna S. Neurofibrosarcoma of lacrimal gland. Indian J Ophthalmol 1987;35:44-8
|How to cite this URL:|
Pattanayak S P, Mathur J S, Thakur V, Khanna S. Neurofibrosarcoma of lacrimal gland. Indian J Ophthalmol [serial online] 1987 [cited 2013 Jun 20];35:44-8. Available from: http://www.ijo.in/text.asp?1987/35/1/44/26316
Neurofibroma of lacrimal gland and its malignant transformation has not been reported as an isolated anomaly. Neurofibromas in the orbit have a bizarre presentation with an incidence of about 2-5% ,, amongst all the orbital tumours. Surprisingly neurofibroma in the orbit as an associated feature of Von Recklinghausen's disease is rare . Isolated neurofibroma of the lacrimal gland has not been reported to the best of our knowledge.
A rare case of neurofibroma of lacrimal gland transforming to neurofibrosarcoma is reported in this communication.
| Case report|| |
R.N., 31 years male patient reported to us with complaints of painless slowly progressive protrusion of the right eye ball of 2 years duration and it had a relatively rapid course for three months, and was associated with marked diminution of vision. There was no history of any injury or fever (photograph 1).
| Clinical examination|| |
General Examination showed no abnormality. Vision in right eye 6/60 and left eye 6/9. Right eye showed slight drooping of the upper eye lid. The eye ball was moderately proptosed and deviated downwards and medically. There was gross, restriction of ocular movements, in upward, up and out and outward gaze. Rest of the ocular movements also were moderately restricted, conjunctiva of the right eye (both palpebral and bulbar) showed mild passive congestion. Fundus examination of the right eye revealed slight blurring of the optic disc margins. There was mild venous dilatation with no foveal reflex.
A freely mobile globular mass (3 cm x 2 cm) with irregular surface and firm consistency was felt in the superior temporal aspect of the right orbit. It was not tender, nonpulsatile and not expansile on coughing. Left eye showed no positive findings on clinical examination.
| Investigation|| |
Complete haemogram including ESR, urine, stool analysis were done and they were found to be within normal limit. X-ray of the skull and orbit showed moderate enlargement of the supertemporal aspect in the right orbit with a soft tissue shadow, in that region. X-ray chest was normal.
Anterior orbitotomy was done under G.A. in the right eye and a globular, moderately vascular and well capsulated mass with two small peduncles on the surface was removed. It measured 3z cm x 31 cm x 3 cm and weighed 13 grams (Photo 2 &3).
| Discussion|| |
Most epithelial tumours seen in the lacrimal gland are benign mixed cell tumours. Adenocystic carcinoma (cylindroma) and rarely adenocarcinomas, squamous cell carcinoma, undifferentiated carcinoma and mucoepidermoid carcinomas. Occasionally, benign mixed cell tumour may undergo malignant transformation. Most non-epithelial involvements of the lacrimal gland are in the form of pseudo tumours, sarcoidosis, lymphoepithelial lesions in Sj φgren's syndrome or in Mikulicz syndrome, and various lymphoid lesions both benign and malignant.
Neurofibroma, is a very rare tumour of the lacrimal gland.
These tumours are chiefly composed of proliferating Schwann cells of peripheral nerve within their nerve sheath and show bundles of wavy eosinophilic fibres with oval nuclei extending in different directions in the connective tissue (H.E.Stain). To our knowledge, malignant transformation of such tumours in the lacrimal gland has never been reported even though sarcomatous transformation of neurofibroma in patients with Von Recklinghausen's disease has rarely been reported ,.
| References|| |
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|5.||Chaudhari, S.: Ronar, S.G. and 11analigod, J.R., 1980, Cancer, 46 : 605. |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]