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ARTICLES
Year : 1973  |  Volume : 21  |  Issue : 1  |  Page : 19-22

Neurofibromatosis with meningioma orbit (a case report)


1 Department of Ophthalmology, Postgraduate Instiute of Medical Education and Research, Chandigarh, India
2 Department of Otolaryngology, Postgraduate Instiute of Medical Education and Research, Chandigarh, India

Correspondence Address:
I S Jain
Department of Ophthalmology, Postgraduate Instiute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


PMID: 4210053

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How to cite this article:
Jain I S, Nagpal K C, Arora M. Neurofibromatosis with meningioma orbit (a case report). Indian J Ophthalmol 1973;21:19-22

How to cite this URL:
Jain I S, Nagpal K C, Arora M. Neurofibromatosis with meningioma orbit (a case report). Indian J Ophthalmol [serial online] 1973 [cited 2020 Nov 26];21:19-22. Available from: https://www.ijo.in/text.asp?1973/21/1/19/31429


  Introduction Top


GASS' stated that von Recklinghau­sen's disease is characterised by (1) neurofibromatosis, (2) cafe au lait spots (3) osseous malformation and (4) gliomas of the brain and optic nerve. The disease can affect the cen­tral and peripheral nervous systems, which may be involved in a variety of neural hamartomas including neurofi­bromas, neurilemmomas, neuronomas, astrocytomas, and meningiomas.

Association of neurofibromatosis and meningiomas was observed by RODRIGUEZ AND BETHRANG. [2] WALSH AND HoYT [3] perhaps reported the only case of meningioma confined to the orbit in a case of generalised neuro­fibromatosis.

This rare association of meningioma with neurofibromatosis has prompted us to report a case of generalised neu­rofibromatosis associated with orbital meningioma. This to our knowledge is perhaps the first to be reported from our country.


  Case Report Top


Darshan Singh, a 12 year old male, presented to the Eye Outpatient De­partment on 24th June, 1970, with the complaint of progressive bulging of his left eye for the last two years. No history of pain, redness, watering or photophobia was recorded. Ocu­lar examination showed visual acuity right eye and left eye 6/12. The left eye was proptosed and it was pushed down and out. No chemosis of con­junctiva was present. The left eye was divergent about 25 0 . Pupil reacted normally. Fundus showed a transversly oval disc. The exophthalmos was non nulsatile, non tender and slightly reducible. Eye movements were full. A mass was palpable in upper and inner quadrant of the orbit, firm, non tender adhe­rent to the underlying structures. The skin over it was freely mobile. Tem­perature was not raised. The left preauricular gland was enlarged.

Systemic examination

One small swelling in the left zygo­matic region on the face was detected. This was in relation to skin and moved with the movement of the skin, non­tender, non adherent to the under­lying structures.

Similar multiple swellings detected all over the body over the arms, back and trunk.

X-ray

X-ray examination of the orbit and paranasal sinuses was normal.

Clinical Impression

Neurofibromatosis with exophthal­mos due to orbital neurofibroma.

On 6th July, 1970, anterior orbito­tomy was done in the upper inner quadrant where an incision was made just below the eye brow region. A tumour of the size of 1'/2 x 1 cm. gre­yish in colour was removed and sent for histopathology.

Biopsy report of the section show­ed clumps and infiltrating streaks of tumour cells organised in a vasofor­mative manner.

The cells formed distinct whorls in some places. The tumour cells were fairly large with eosinoohilic cyto­plasm and resembled meningothelial cells [Figure - 1],[Figure - 2]. The morpholo­gical impression was meningothelial meningioma.

Skin nodule biopsy was done on 20th July and confirmed the clinical diagnosis of neurofibromatosis. All the nerves included in the biopsy re­vealed a concentric, proliferation of the connective tissue [Figure - 3],[Figure - 4].

Final diagnosis

Generalised neurofibromatosis with meningothelial meningioma left orbit with exophthalmos left eye.


  Discussion Top


GASS[1] defined hamartoma as a tumour of anomalous or malformed tissue composed of elements normal to the organ or area where it arises. He included many tumours under the heading neural hamartoma like neuro­fibromas, neurilemmoma, neuronomas, astrocytomas and meningiomas. He also advocated the term hereditary hamartomatosis for all the syndromes included under the term phakomato­sis and stated that in these syndromes not only the clinical picture may be incomplete but also a mixture of clini­cal features of several syndromes may be present in a single patient or in members of the same family.

The present case was a typical example of the mixture of the hamar­tomas. There were neurofibromas scattered all over the body and in as­sociation there was meningothelial meningioma of the left orbit. It was interesting to note that the case ob­served by WALSH AND HOYT [3] also had meningothelial meningioma of the or­bit in association with generalised neurofibromatosis. Miningothelial meningiomas are slow growing turn­ours and are characterised by forma­tion of psammoma bodies which re­sulted from hyalinisation of the intima of blood vessels joined together with the contained blood and followed by calcification. Our case showed clumps and streaks of tumour cells organised in a vasoformative manner. The cells formed distinct whorls in some places. The tumour cells were fairly large with eosinophilic cytoplasm and re­sembled meningothelial cells [Figure - 1],[Figure - 2].

Clinical diagnosis of meningioma was difficult because of the rarity of orbital meningioma itself and further rarer association of neurofibromatosis with meningiorna of the orbit. Hence in such cases the diagnosis must await surgical exploration and histopatholo­gical examination. It signifies that amongst the causes of exophthalmos apart from neurofibromas of the orbit and gliomas of optic nerve, an orbital meningioma should be a distinct pos­sibility.


  Summary Top


A case of neurofibromatosis with meningioma of the orbit is reported. Amongst the causes of exophthalmos in cases of neurofibromatosis, apart from neurofibroma of orbit and glioma of optic nerve, a meningioma should be kept as a distinct possibility. It had no relationship with the sheaths of the optic nerve.

 
  References Top

1.
Gass, J. D. M.: The phakomatoses in Neuro-ophthalmology. Sympo­sium of the University of Miami and the Bascam Palmer Eye Insti­tute. Edited by J. Lawton Smith: The C. V. Mosby Company Vol. II, pp. 223-46, St. Louis, 1965.  Back to cited text no. 1
    
2.
Rodriguez, H. A. and Betherang, M.: Multiple primary intracranial tumours in von Reckinghausen's neurofibromatosis. Arch. Neurol. (Chicago) 14: 467-477, 1966.  Back to cited text no. 2
    
3.
Walsh, F. B. and Hoyt, W. F.: Cli­nical Neuro-ophthalmology Vol. III. Third Ed. pp. 2080, The Williams and Wilkins Company Baltimore, 1969.  Back to cited text no. 3
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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Introduction
Case Report
Discussion
Summary
References
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