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| ARTICLES |
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| Year : 1976 | Volume
: 24
| Issue : 3 | Page : 1-5 |
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Peripheral nerve tumours of the orbit
R Gogi, K Nath, AA Khan, S Hameed
Institute of Ophthalmology J. N. Medical College Aligarh, India
Correspondence Address:
R Gogi
Institute of Ophthalmology J. N. Medical College, Aligarh
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 1031397 
How to cite this article:
Gogi R, Nath K, Khan A A, Hameed S. Peripheral nerve tumours of the orbit. Indian J Ophthalmol 1976;24:1-5 |
Peripheral nerve tumours of the orbit include neuro-fibroma, neurilemoma, plexiform neuroma, neuroma and some rare tumours like neurogenic sarcoma, granular cell myoblastoma and non-chromaffin paraganglioma[7].
Neurofibroma is a benign encapsulated tumour arising from nerves and permeated by nerve fibres[4]. Generaly, the tumour is a local manifestation of von. Recklinghausen's diseases. Such tumours in the orbit are rare[4]. The incidence of neurofibroma amongst all orbital tumours is about 3%[20].
It manifests itself in childhood[20]. No specific relationship with sex has been observed and causes proptosis of gradual onset. In advance cases, the intracranial pulsations may be transmitted into the orbit, due to the destruction of the orbital bones. Associated pigmented spots on the skin are present[20].
Plexiform neuroma is not a common orbital tumour. In neurofibroma, some proliferation occurs within the sheath leading to marked enlargement and tortuosity of the nerve. When the tumour can be palpated beneath the skin as a vermiform cord, it is labelled as a plexiform neurofibroma[7]. In advanced cases degeneration with deposition of gelatinous tissue may occur[4].
Neurilemoma is a benign tumour which usually arises from the acoustic nerve near the internal auditary meatus[29]. Tertsch[27] reported a case of neurinoma of orbit which he believed to be the first on the record. This was followed by two reports by Marchetti[10] and Parkar[18]. The first typical histological picture of the tumour was described by Cohan[2]. Das[3] reported eight cases of neurilemoma from India. Neurilemoma of orbital bones is a very rare type of bone turnout[14]. The recurrence of this tumour is uncommon[29]. Nath and Gogi[16] reported a case which recurred after three years.
| Material and Methods | |  |
In a study of 120 consecutive cases of histologically proved primary orbital tumours eleven cases of peripheral nerve tumours were seen. Their clinicopathological findings are hereby presented.
| Observations | | |
1. Neurofibroma :
Three cases of neurofibromas, occuring in two males and one female, were seen in their first, sixth and third decade respectively. Clinically none was associated with generalized neurofibromatosis. All the three had unilateral proptosis while one was associated with a neurofibroma of the upper lid.
Gross Appearance : In two cases the tumour mass measured 1.5 X 0.5 x 0.5 cm [Figure - 1] . and firm. In the cm. Tumours were smooth and 2.5 x 2.0 x 2.0 third case, who was associated with neurofibroma of the lid, the cord like nerves could be palpated in the swelling.
Microscoppic Appearance : Microscopic section showed proliferation of nerve fibres arranged haphazardly, separated by elongated cells and collagenousfibres [Figure - 2].
Neurilemoma :
This tumour was seen in seven cases, affecting four females and three males aged, between 8 and 80 years. Clinically the tumour was slowly growing and caused unilateral progressive proptosis. In one case the tumour recurred after three years of its first removal.
Gross Appearance : All the tumours were encapsulated, smooth greyish white and firm in consistency. The size varied from 1.0 x 1.0 x 0.5 surface was smooth and dull cm [Figure - 3]. Cut cm to 3.5 x 3.0 x 1.5 white.
Microscopic Appearance: The following two types of histological patterns were observed in neurilemomas :
(a) Tumours were encapsulated with elongated or spindle shaped cells arranged in a palisade manner with a cellular area in between [Figure - 4]. This was neurilemoma Antoni type-A and such six cases were observed.
(b) In the second type the cells were irregularly arranged with numerous microcystic spaces in between. This was neurilemoma Antoni type-B, and one such case was recorded.
Plexiform Neuroma : One case of plexiform neuroma was observed in an eleven years' old female child. Vermiform swellings involving the upper lid and the orbit could be palpated.
Gross Appearance : There were five cord like creamy white swellings approximately 1.0 to 2.5 cm long and 0.5 to 1.0 cm thick bunched together in connective tissue.
Microscopic Appearance : It consisted of nerve bundles of variable sizes showing marked proliferation of nerve fibres [Figure - 5],[Figure - 6] with areas of degeneration.
Comments
There are eleven peripheral nerve tumours in this study, from a series of 120 primary orbital tumours. The incidence of 9.16% in this study falls close to that reported by other workers[3],[6],[12],[24]. The peripheral nerve tumour group comprises of three cases of neurofibroma, seven of neurilemoma and one of plexiform neuroma.
The neurofibromas were localized to the orbit and the upper lid without any evidence of neurofibromatosis elsewhere. In this study, the neurilemomas were responsible for 5.8% of primary orbital tumours while in another series the incidence of this tumour was 7.49%[3]. However, a low incidence of this tumour (1%) has been recorded by the other workers[1],[6],[8],[17],[20],[22]. The tumour can occur in either sex at any age. It is a benign nerve sheath tumour which grows slowly and possibly intermittently[20]. Due to its pressure upon the optic nerve it may cause blurring of the vision. Rarely it may present with panophthalmitis[21], or as a case of retrobular neuritis[13]. There may be pigmentation of the skin in cases of neurilemoma which indicates the close relationship of this tumour with von Recklinghausen's disease[15],[20],[25]. However, none of these findings were noted in any of our cases.
Histogenesis
There is some uncertainty about the precise cell of origin of neurilemoma. This accounts for the use of different nomenclature by different workers. Neurinoma[28] indicating nerve fibretumour, perineural fibroblastoma[9],[19] are evidently inappropriate. The names Schwannoma[11] and Neurilemoma[26] are commonly accepted which indicated its precise sheath origin from Schwann cells.
Neurofibromas arise as a result of diffuse proliferation of the Schwann cells of the peripheral nerve and involve fibroblastic elements of endo-neurium and perineurium[5].The whole complex of neurofibromatosis is viewed as a harmartoma[7],[20]. The plexiform neuroma is a variant of neurofibroma, where there is marked proliferation of nerve trunk within the sheath.
| Summary | | |
1. From a study of 120 consecutive cases of primary orbital neoplasms, eleven cases of peripheral nerve tumours have been described.
2. There are seven cases of neurilemoma, three cases of neurofibroma one case of plexiform neuroma.
3. Histopathological findings in these cases have been described in detail.
| Acknowledgement | | |
We are thankful to Mr. A.Y. Khan, senior technician of Institute, for processing the tissue for histopathological examination. We are also grateful to Mr. U.C. Gupta, senior phototechnician of Institute for carrying out photography work for us.
| References | | |
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]
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