|Year : 1973 | Volume
| Issue : 1 | Page : 19-22
Neurofibromatosis with meningioma orbit (a case report)
IS Jain1, KC Nagpal1, M.M.L Arora2
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
I S Jain
Department of Ophthalmology, Postgraduate Instiute of Medical Education and Research, Chandigarh
|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 2013 May 26];21:19-22. Available from: http://www.ijo.in/text.asp?1973/21/1/19/31429
| Introduction|| |
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.  WALSH AND HoYT  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|| |
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.
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 examination of the orbit and paranasal sinuses was normal.
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].
Generalised neurofibromatosis with meningothelial meningioma left orbit with exophthalmos left eye.
| Discussion|| |
GASS 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  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|| |
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|| |
|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. |
|2.||Rodriguez, H. A. and Betherang, M.: Multiple primary intracranial tumours in von Reckinghausen's neurofibromatosis. Arch. Neurol. (Chicago) 14: 467-477, 1966. |
|3.||Walsh, F. B. and Hoyt, W. F.: Clinical Neuro-ophthalmology Vol. III. Third Ed. pp. 2080, The Williams and Wilkins Company Baltimore, 1969. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]