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ARTICLES
Year : 1971  |  Volume : 19  |  Issue : 1  |  Page : 27-30

Myxosarcoma of the orbit


Department of Ophthalmology, Post-Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
J S Gupta
Department of Ophthalmology, Post-Graduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


PMID: 15744961

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How to cite this article:
Gupta J S, Gopal H, Dhavan S K. Myxosarcoma of the orbit. Indian J Ophthalmol 1971;19:27-30

How to cite this URL:
Gupta J S, Gopal H, Dhavan S K. Myxosarcoma of the orbit. Indian J Ophthalmol [serial online] 1971 [cited 2020 Feb 27];19:27-30. Available from: http://www.ijo.in/text.asp?1971/19/1/27/35000

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 [1] reported a case of recurring myxosarcoma of the orbit in a 38 years old female. Strickland [2] 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 Top


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.

Investigations:

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.

Operations Notes:

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 Top


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 Top


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 Top

1.
BUCKLEY J. H.: Recurring myxo­sarcoma of orbit, Amer. J. Ophthal. , 207, (1922).  Back to cited text no. 1
    
2.
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)  Back to cited text no. 2
    


    Figures

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



 

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