Malignant schwannoma of orbit

Vidya Kachole; DL Maria; A Khalique; BR Solanki

Home

Users Online: 1719

ARTICLES

Year : 1979 | Volume : 27 | Issue : 1 | Page : 43-45

Malignant schwannoma of orbit

Vidya Kachole¹, DL Maria¹, A Khalique², BR Solanki²
¹ Department of Ophthalmology, Medical College, Aurangabad, India
² Department of Pathology, Medical College, Aurangabad, India

Correspondence Address:
Vidya Kachole
Department of Ophthalmology, Medical College, Aurangabad
India

Source of Support: None, Conflict of Interest: None

Check

PMID: 500181

How to cite this article:
Kachole V, Maria D L, Khalique A, Solanki B R. Malignant schwannoma of orbit. Indian J Ophthalmol 1979;27:43-5

How to cite this URL:
Kachole V, Maria D L, Khalique A, Solanki B R. Malignant schwannoma of orbit. Indian J Ophthalmol [serial online] 1979 [cited 2021 Mar 9];27:43-5. Available from: https://www.ijo.in/text.asp?1979/27/1/43/31547

Schwannoma of orbit is a rare tumour. Its malignant variety, malignant schwannoma (Neurogenic Sarcoma, Malignant neurilemmoma fibrosarcoma of the nerve sheath) is more rare[3]. Instances of malignant schwannomas of orbit have been reported by Calvest and Canadau[4], Cerveira[2], Ginde[5] Schiff-Wertheimer and Loisillier[12], Reese[9] Radnot[10], Emilier[4] and Mortada[8]. Schatz[11] has reported a case where typical benign lesion after presumably incomplete removal underwent sarcomatous transformation.

Case Report

A 28 years old male patient was admitted in Ophthalmic ward of Medical College, Aurangabad with the complaint of protrusion of left eye for one month. The past, personal and family history did not reveal any significant information. On admission there was unilateral axial proptosis with slight chemosis and restriction of movements of the eyeball. There was no other positive finding. Visual acuity in both eyes was 6/9 and fundi were normal [Figure - 1]

One week after the admission the visual acuity in left eye was 6/12 and there was evidence of early papilloedema in the form of hyperemia of optic disc and venous fullness. Proptosis was same.

In further two weeks the vision deteriorated rapidly and visual acuity was reduced to 3/160. The papilloedema was frank and chemosis had also increased. Proptosis still remained same.

Investigations

Total haemogram, stool and urine examination did not reveal any abnormality. Post meal blood sugar level was 84 mg.%. Serum K.T. and V.D.R.L. test was negative. X-Rays of skull, orbit and chest were normal. The ear, nose, and throat examination showed deviated nasal septum to the right side.

Treatment

In the beginning the patient was put on antibiotics and corticosteriod to rule out the possibility of pseudo tumour. But there was no response to this treatment. Instead the visual acuity became less. Then Kronleins' orbitotomy was done and a firm mass was removed in two pieces which was surrounding the optic nerve and the proptosis subsided [Figure - 2].

Histopathological examination showed a tumour formed by plump spindle shaped cells. Some of the cells were oval. No pleomorphism was observed. Nuclear pallisading and arrangement of cells in groups was seen [Figure - 3][Figure - 4]. On examination all these cells of tumour showed features of malignancy in the form of anaplasia and hyperchromicity of nuclei. Hence the diagnosis as malignant Schwannoma was made.

Patient was then referred to Tata Memorial Cancer Hospital, Bombay for advice.

Discussion

Schwannoma is the benign tumour occuring any where along peripheral, cranial or sympathetic nerves[8]. Orbit is a rare site. Malignant tumour may be primarily arising in the orbit or following incomplete removal of benign schwannoma of the orbit as reported by Schatz[11]. It may arise in neurofibromatosis[6],[8]. Approximately one half of the malignant schwannomas arise in patients who have von Recklinghausen's disease.

Schwannoma arises from the Schwann cells of the nerve sheath. Histopathologically the malignant Schwannoma is highly cellular tumour consisting of plump spindle shaped or oval cells generally showing little cellular pleomorphism and often is accompanied by collagen fibre. Nuclear pallisading as well as an arrangement of cells in groups, nests, cords, or whorls are diagnostic features. Distant metastasis are frequent in highly cellular form of this tumour.

It is a radioresistant tumour. In the majority of the cases studied by Reese[9] the disease was fatal.

Summary

A rare malignant retrobulbar tumour of orbit confirmed histopathologically as a malignant Schwannoma in a young male is being reported.

References

1.	Calvet and Canadau, 1949, System of Ophthalmology, 13, Part 11, Lacrimal orbital and paraorbital diseases, 1106, 1974, Henry Kimpton, London.
2.	Cerveira, 1953, System of Ophthalmology, 13, Part II, 1106, 1974, Henry Kimpton London.
3.	Duke Elder, S., 1974, System of Ophthalmology, 13, 1106, Henry Kimpton, London.
4.	Emilier, 1965, System of Ophthalmology, 13, 1106, 1974, Henry Kimpton London.
5.	Ginde, 1953, Tumours of the Eye by Reese, 2nd Edn. 202, 1963, New York.
6.	Hogan M.J. and Simmerman L.E., Ophthalmic pathology An Atlas and Text Book, 2, 755, Saunders, Peiladelphia and London.
7.	Hoses, 1931, System of Ophthalmology, 13, Part II 1106, Henry Kimpton London.
8.	Mortada, 1968, Brit. J. Ophthal, 52, 188, 550
9.	Reese, 1963, Tumours of Eye, 2nd Edn. 199, New York.
10.	Rodnot, 1963, System of Ophthalmology, 31, Part 11 1106, 1974, Henry Kimpton London.
11.	Schatz (1971) Arch. of Ophthal, 86, 208.
12.	Schiff Wartheimer and Loisillier, 1954, Tumours of Eye by Reese, 2nd Edn. 201, 1964, New York.