|Year : 1971 | Volume
| Issue : 1 | Page : 27-30
Myxosarcoma of the orbit
JS Gupta, Hari Gopal, SK Dhavan
Department of Ophthalmology, Post-Graduate Institute of Medical Education and Research, Chandigarh, India
J S Gupta
Department of Ophthalmology, Post-Graduate Institute of Medical Education and Research, Chandigarh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta J S, Gopal H, Dhavan S K. Myxosarcoma of the orbit. Indian J Ophthalmol 1971;19:27-30
Myxosarcoam of the orbit, arising in the soft tissues immediately behind the eye, is an extremely rare malignant tumour. The tumour may arise from the cell rests of embryonic tissue or it may be a myxomatous degeneration in an adult type of sarcoma. Buckley  reported a case of recurring myxosarcoma of the orbit in a 38 years old female. Strickland  described a radiation induced fibromyxosarcoma of the orbit 33 years after treatment of bilateral ocular glioma treated at the age of 15 months by a combination of surgery and radium. The present case is being presented to highlight some unusual features.
| Case Report|| |
H. S., a 2 years old male child, presented to us on 8th April, 1968 with a short history of 10 days when the parents noted a "swelling" of the left eye in the morning. The child was otherwise well, eating and playing as usual. The swelling increased rather rapidly and painlessly into its present size. There was no history of injury, fever, vomiting or any other ailment. He was the second born child. The first one died soon after birth, the cause for which could not be ascertained. Birth history and family history were non-contributory.
On examination the child was moderately built and nourished, co-operative and active. Body temperature was normal. Cardiovascular system, chest and abdomen were normal. There was no lymphadenopathy; no abnormality or tenderness of bones.
Ocular examination revealed a straight, axial proptosis of the left eye (first e xophthalmometry reading 32 mm.) with exposure keratitis. The lids were essentially normal but stretched due to proptosis. The conjunctiva was more chemosed than congested and showed signs of drying in the interpalpebral region. The anterior chamber was normal, pupil was mid-dilated and fixed but not leucokoric. Ocular movements were restricted in all directions. Fundus could not be seen and no visual assessment was possible.
On palpation the orbital margins were normal, the proptosis was irreducible, nonpulsatile and non-tender. There was no fullness of the orbital spaces on pressing through the lids and no definite mass could be detected.
The right eye was essentially normal.
E.N.T. check up -
nothing abnormal was detected.
X-ray skull, optic foramina and chest were normal.
Blood picture: Hb 7.25 gm.%
E.S.R. 22 mm/first hr.;
Total leukocyte count was 10,000/c. mm. and the differential count was neutrophils 59%, lymphos 40%, basophils 1%, monocytes and eosinophils nil.
No abnormal or immature cells were seen.
Urine and stools, nothing abnormal was detected.
Patient was operated on 11-41968 under general anaesthesia. Through the conjuctiva, lateral orbitotomy was done. On exploration of the orbit it was felt that the optic nerve was slightly thickened and a definite soft to firm mass was felt in the posterior orbital region. Thus, the eye ball was enucleated including the optic nerve as far behind as possible. The orbital mass was then removed as thoroughly as possible. There was very little bleeding throughout the operation. Naked eye observation revealed an intact, normal size eye ball but slightly thickened nerve especially near its entry into the sclera. The orbital mass was pearly pink, friable and slippery.
The tumour consists of spindle/stellate cells in whorls and bands, with loose stroma which is often myxoid. Tumour is malignant and shows potentiality of a tumour arising from embryonic connective tissue. Section from the eye ball shows infiltration along the optic nerve [Figure - 1]. No intraocular pathology seen.
Histopathological diagnosis was embryonal sarcoma with characters of myxosarcoma.
Postoperative Follow Up:
Within three days the orbital growth recurred so fast as to give a protrusion almost equal to the original proptosis [Figure - 2].
Deep X-ray therapy was started on 15th April, 1968 and a total dose of 3,000 r was given in 14 sittings. The tumour mass regressed completely and the patient was discharged from the hospital [Figure - 3].
| Discussion|| |
A short history of ten days with rapid unilateral proptosis were highly suggestive of inflammation but an afebrile course and a normal blood picture ruled out acute infection. Sarcomas, especially arising from embryonal tissues are known to be highly rapid in development producing an extreme degree of proptosis, simulating an orbital inflammation. Due to nonencapsulated nature of the tumour it was not possible to remove it completely so there was rapid recurrence of the tumour in the postoperative period but fortunately deep X-ray therapy was very effective. Inspite of the rapid growth of myxosarcoma in the present case, no distant metastasis were seen. However, an unusual feature was infiltration along the optic nerve by the malignant sarcomatous cells which has not been reported so far.
| Summary|| |
A case of orbital myxosarcoma in a 2 years old male child showing highly rapid growth, extreme proptosis and sarcomatous infiltration of the optic nerve is reported.
| References|| |
BUCKLEY J. H.: Recurring myxosarcoma of orbit, Amer. J. Ophthal. , 207, (1922).
STRICKLAND P:. Fibromyxosar coma of the orbit. Radiation induced tumour 33 years after treatment of bilateral ocular glioma, Brit. J. Ophthal., 50, 50-53, (1966)
[Figure - 1], [Figure - 2], [Figure - 3]