|Year : 1979 | Volume
| Issue : 3 | Page : 49-51
Neurilemmoma of orbit
SC Sen1, NC Pal2, AK Das3
1 Department of Ophthalmology, R.G. Kar Medical College Hospital, Calcutta, India
2 Department of Pathology, R.G. Kar Medical College Hospital, Calcutta, India
3 Institute of Post-Graduate Medical Education & Research, Calcutta-700020, India
S C Sen
Department of Ophthalmology, R.G. Kar Medical College Hospital, Calcutta
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sen S C, Pal N C, Das A K. Neurilemmoma of orbit. Indian J Ophthalmol 1979;27:49-51
Neurilemmomas are benign localised, encapsulated tumours arising from the Schwann's cells of peripheral, cranial and sympathetic nerves as well as from the spinal nerve roots,,,. Of the cranial nerves, the auditory nerve is a common site of involvement, the trigeminal accounts for a few, They also involve ciliary nerves in the orbit,. Neurilemmoma of the orbit grows very slowly and possibly intermittently and has no distinct clinical feature. When large enough it causes exophthalmos, limitation of ocular movements and blurring of vision through pressure on optic nerve. Rarely orbital bones are involved with neurilemmoma. Malignant changes are very rare,,. A few cases of Neurilemmoma have been reported from India,,,,,,. Apart from proptosis, orbital neurilemmoma may rarely present as a case of panophthalrnitis. Because of its rare incidence, a case of orbital neurilemmoma, presenting as panophthai-mitis is being reported.
| Case report|| |
S. L., a female aged 13 years was admitted in Eye ward of Bankura Sammilani Medical College Hospital with the complaints of gradual protrusion of the left eye for about one and half years, loss of vision in left eye for about one year, and pain in left eye and left side of forehead for two months. There was no history of trauma in that eye.
On examination of the left eye-the lids were swollen. Conjunctival congestion and chemosis were marked. There was moderate proptosis with lagophthalmos, resulting in sloughing corneal ulcer with prolapse of uveal tissue in the central region. The eye was displaced forward, laterally and slightly downwards with total limitation of all movements. There was no perception of light and was diagnosed as panophthalmitis. A firm mass was palpated on the medial side of the eye ball. The posterior limits of the mass could not be defined. The growth was free from adjacent orbital bones and was not tender. The preauricular and submandibular lymph glands were not enlarged on left side. Right eye was normal. Systemic examination was normal.
Investigations-Haemogram, urine, VDRL test were normal. X-rays of skull, orbits and paranasal sinuses showed no abnormality. Optic foramina were normal.
The left eye along with the adherant growth was enucleated under general anaesthesia. The optic nerve was cut as far behind as possible.
The growth was attached to the postero-medial surface of the eye ball, extending medially upto the insertion of medial rectus, [Figure - 1]. The extent of the growth on the upward, lateral and downward aspects was between 16 to 18 mm from the limbus. It was encapsulated, nodular, firm, irregularly oval and bluish white in colour. The size of the growth was 4 cm x 2.5 cm x 2.2 cm. Feel of the eyeball was firm. Cornea was ulcerated with a big central perforation and prolapse of uveal tissue. Cut section showed solid greyish-white tumour with central mottled haemorrhagic necrosis. The tumour was continuous with upper surface of the optic nerve upto 7 mm from its attachment to sclera. The tumour also extended inside the eye and occupied about 2/3rd of intraocular space posteriorily. The uveal tissue was compressed and pushed forward and downward, lens was absent.
Histopathology-Multiple sections were taken from the different parts of the tumour including the intraocular mass. Considerable thick fibrous capsule was noted at the outer side of the tumour mass. Section from the one extreme corner of the tumour mass showed presence of a nerve trunk [Figure - 2]. The tumour cells were relatively uniform in appearance and at different areas showed characteristic palisading arrangement of nuclei [Figure - 3]. At many places tumour cells showed disorderly arrangement and often with intracellular vacuoles. All throughout, the tumour tissue showed few well formed blood vessels.
The post-operative period was uneventful. The patient was followed up for about two years and there was no recurrence of the growth.
| Discussion|| |
Though there wits proptosis and loss of vision, the patient did not attend hospital earlier due to lack of facilities. She attended hospital only when there was severe pain in that eye due to complicated corneal ulcer. Swollen lids, proptosis with sloughed corneal ulcer gave the appearance of panophthalmitis.
Neurilemmorna has got no distinct clinical feature. So the diagnosis depends solely on the characteristic histological findings,- the pallisaded pattern due to alternation of fibrous and nuclear zones,. In the present case the growth was solitary and was unaccompanied by signs of neurofibromatosis or other lesion of the nervous system and had the characteristic palisading arrangement of the nuclei. The tumour was demonstrated to arise on the course of a nerve, [Figure - 2], presumably a ciliary nerve, at the site favoured by benign schwannomas. These facts established the diagnosis of neurilemmoma beyond doubt in this case.
| Summary|| |
A case of neurilemmoma of orbit presenting as a case of panophthalmitis in a young girl has been reported. The criteria for its diagnosis histologically has been discussed.
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[Figure - 1], [Figure - 2], [Figure - 3]