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   Table of Contents      
Year : 1973  |  Volume : 21  |  Issue : 2  |  Page : 56-58

Peripheral nerve tumors of the eye

1 Department of Pathology, Andhra Medical CollegeVisakhapatnam, India
2 Department of Ophthalmology, Andhra Medical College, Visakhapatnam, India

Correspondence Address:
C.R.R.M Reddy
Department of Pathology, Andhra Medical CollegeVisakhapatnam
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Source of Support: None, Conflict of Interest: None

PMID: 4789111

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How to cite this article:
Reddy C, Chetty G R, Sundareswar B. Peripheral nerve tumors of the eye. Indian J Ophthalmol 1973;21:56-8

How to cite this URL:
Reddy C, Chetty G R, Sundareswar B. Peripheral nerve tumors of the eye. Indian J Ophthalmol [serial online] 1973 [cited 2020 Dec 2];21:56-8. Available from: https://www.ijo.in/text.asp?1973/21/2/56/31411

Peripheral nerve tumors usually occur in relation to large nerves and of flexor aspects of the limbs. Face, neck, scalp, hands and feet are not often involved (RUSSELL [2] ). In Stout's series no tumors are seen in the feet, urogenital organs, lungs, esophagus and rectum (WILLIS [3] ). Eye involve­ment in neurofibromatosis is more common than the clinical examination indicates but neurinomas of the eye are uncommon (REESE [1] ). Every part of the eye, including lids and adnexae may be involved in cases of generalised neurofibromatosis. The neurofibromas have distinctive clinical features whereas the neurinomas have no distinctive clinical features.

While reviewing a large number of peripheral nerve tumors we came across five peripheral nerve tumors of the eye and because of their rarity, they are presented below along with a detailed case report of a neurinoma of the orbit.

  Case Report Top

A female aged 55 years was admitt­ed with protrusion of the right eye ball and pain in the right eye of 5 months' duration. The protrusion was gradually increasing. A swelling was present on the medial canthus since 5 months causing the protrusion of the eye ball. There was another small swelling since 2 months on the upper and lateral aspect of the same eye.

General examination did not reveal any significant finding. There were no cafe-au-lait spots nor any other swellings.

Local Examination

Position of the right eye ball was down and out. Eye ball movements up and in, and up and out were restricted, the other movements being normal. The swelling above the medial canthus was 3 x 1 cm. in size, smooth, not mobile, firm and the borders were delineated all round except on the temporal side [Figure - 1]. The swelling was not painful on pressure. The swelling on the outer and upper aspect of the eye ball was 1 x 1 1/2 cms. bosselated and firm and could be pushed into the orbital cavity but would come out on releasing the pressure. It was not tender and it could be seen on the conjunctival surface protruding on the surface of the eye ball. No pulsations of the globe could be felt.


The lids were not covering the eye ball completely and a scleral strip of 5 mm. could be seen beneath the cornea and margin of the lower lid, when the lid was closed.

Both eyes were aphakic. The right cornea was hazy in the lower half. The left cornea was normal. Tension was 14.5 mm of Hg in the left eye and 15.5 mm of Hg. in the right.


Blood group `O'. Blood pressure: 128/80 mm of Hg.

Hemoglobin 9 -gms%; Total W.B.C. Count 9500/cm.

Differential Count: Polymorphs 64, Eosinophils 4, Lymphocytes 30, Large mononuclears 2. E.S.R.: 30 mm/hr.

X-ray Orbit: Enlargement of right orbit seen.

X-ray of optic foramen, antero­posterior and lateral view of the skull and of the clinical nasal sinuses showed no abnormality clinical examination of the nose revealed no abnormality.

Biopsy Report of the removed piece of the material received showed typical neurinoma with Antoine type A and Antoine type B tissue [Figure - 2]. There was well marked pallisading of the nuclei. The blood vessels showed thickened walls.

  Comment Top

While reviewing 240 cases of peri­pheral nerve tumors from the files of the Department of Pathology, we came across five cases involving the eye. Three of them were neurinomas and the other two neurofibromas.

The three neurinomas were seen in 2 females and one male. The ages were 18, 35, and 55. Right orbit was involved in 2 cases and the left in one case.

In both the cases with neurofibroma the eye lids were involved. Both were boys, one aged 12 and the other 14 years. In both cases there was ptosis and swelling of the eye lid. In one the tumour tissue extended on to the temporal region.

Neurinomas do not occur in any particular age group. The ages may range from 7 to 70 years (Reese [1] ). They have no distinctive clinical features. They progress slowly or inteuuittently and may lie quiescent for long periods. Their size varies. They are usually solitary, well encapsulated and on complete excision the prognosis is excellent. Apart from occurring in the eye neurinomas may occur in lids, conjunctiva, sclera and iris also.

Neurofibromas cause much more clinical symptomatology. There may be asymmetry of the face, cornea may be larger, lids may show ptosis and cafe-au-lait pigmentation may be present. Orbit may show bone des­truction or hypertrophy. When there is bone destruction intracranial pulsa­tions may be transmitted to the eye. The pulsations are synchronous with the pulse, unaccompanied by bruit but there will be no discomfort. Exoph­thalmos may occur as a result of 1. neurofibromatous involvement of the orbit, 2. herniation of brain due to orbital bone erosion and 3. gliomas or meningiomas of the optic nerve which may be associated. Conjunctiva is not often involved. Uvea may be involved in a diffuse manner involving the choroid, ciliary body and iris. Buphthalmos might occur when glaucoma occurs.

The present case of neurinoma belonged to the older age group and showed exophthalmos with limitation of movements of the eye ball.

  Summary Top

A case of neurinoma of the eye is reported with a review of the literature on the tumors of the peripheral nerves of the eye.

  References Top

Reese, A. B.: Tumors of the eye. First Edition P. 176, Paul B. Hoeber, U.S.A., (1951).  Back to cited text no. 1
Russell, D. S., Rubinstein, L. J. and Lumsden, C. E.: Pathology of tumors of the nervous system. First Edition. P. 234, Edward Arnold Ltd., London. (1959).  Back to cited text no. 2
Willis, R. A.: Pathology of tumors. Third Edition. P. 832, Butterworth's. London. (1960).  Back to cited text no. 3


  [Figure - 1], [Figure - 2]


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