|Year : 1992 | Volume
| Issue : 3 | Page : 92-93
Mesenchymal chondro sarcoma of orbit with intra cranial extension (A rare case)
Anila Khuteta, YC Mishra, CL Pandey, PK Mathur, Ravindra Karra
S.M.S. Medical College & Hospital, Jaipur, India
S.M.S. Medical College & Hospital, Jaipur
Source of Support: None, Conflict of Interest: None
A case of Chondro Sarcoma of the orbit with intracranial extension in a 10 year female presented as unilateral proptosis and primary optic atrophy in the left eye and post papilloedema optic atrophy in right eye is being presented.
|How to cite this article:|
Khuteta A, Mishra Y C, Pandey C L, Mathur P K, Karra R. Mesenchymal chondro sarcoma of orbit with intra cranial extension (A rare case). Indian J Ophthalmol 1992;40:92-3
| Introduction|| |
Mesenchymal Chondro Sarcomas are very rare tumours which arise from bone or from soft tissue was first described by Lichtenstein, & Bernstein  in the body. Reese  stated that such tumours usually involve the orbit by extension and rarely arise from within it. We present such a rare tumour arising in the orbit and extending into the middle cranial fossa.
| Case report|| |
A 10 years old girl presented with painless axial proptosis of 8 mm. and gradual loss of vision on the left side for 2 years and gradual and complete loss of vision in the right Eye for 5 months. Left eye movements were restricted in all directions. Conjunctiva was markedly congested and oedematous [Figure - 1]. Fundus showed primary optic atrophy in the left eye and post-papilloedema optic atrophy in the right eye. Roentegenographic examination of the left orbit showed several cloud like calcification characterstic of cartilaginous tissue mass occupying the apex of the orbit with erosion of the superior orbital fissure at the upper margin [Figure - 2] and optic foramen. Lateral view of the skull showed destruction of the pituitary fossa with erosion of the anterior clinoid process.
A provisional diagnosis of glioma of the optic nerve with chiasmal extension was made and exenteration of the left orbit was done to do partial excision of the mass and confirm the diagnosis. During surgery a multilobulated well circumscribed bluish coloured mass was seen at the apex or orbit, the optic nerve was very hard and nodular. The posterior margin of the mass could not be reached as it was extending into the middle cranial fossa. A local excision of the mass was done and sent for histopathology which confirmed the diagnosis of Mesenchymal Chondrosarcoma [Figure - 3].
| Discussion|| |
Chondroma and chondrosarcoma constitute about 10% of all primary osseous tumours . They are locally malignant tumours. They usually arise from the bones adjacent to the orbit, extending to it secondarily but in rare case they are primary in the orbit. Besides the conventional Chondrosarcoma, there are three other main types: Post radiation, Myxoid and extra skeletal Mesenchymal type which tends to occur in the orbit . The first case in the orbital location was described by Raminez .
Guccoin et al reported 10 cases of this unusual but distinct malignant tumour, four of which occurred in the orbit. There are two more reported cases of orbital Chondrosarcoma in literature which brings the total of Primary Orbital Chondrosarcomas to six ,.
Exophthalmos, diplopia, headache and orbital pain occur with the orbital lesion. Reese reported a case in a 9 year old Negro girl with right sided exophthalmos as the result of Chondrosarcoma of the greater wing of the sphenoid which grew forward into the orbit, and posteriorly into the intracranial cavity.
In our case as the history correlates the patient had primary chondrosarcoma in the left orbit with proptosis and loss of vision. After few months she started having headache, vomiting, vertigo with gradual loss of vision in the right eye, which is explained by middle cranial fossa extension, raised I.C.T. and post papilloedema optic atrophy in the right eye.
| References|| |
Lichtenstein, L. and Bernstein, D.; Unusual benign and malignant Chondroid tumours of bone. Cancer 12: 1142-1157, 1959.
Reese, A.B.: Tumours of Eye, page 320, III edition, Harper & Row Publishers 1976 London.
Dahlin, D.C. and Henderson, E.D.: Mesenchymal C.S.: Further observation on a new entity. Cancer 15: 410-417, 1962.
Guccion, J.G.; Font, R.E.; Exizinger, F.M.; and Zimmerman L.E.: Extra Skeletal Mesenchymal Chondro sarcoma. Arch. Pathol.95:336-340, 1973,
Raminez, M.D.: Mesenchymal Chondro sarcoma of orbit. Arch. Ophthal. Vol. 86, 410-413, 1971.
Trzcinska-Dabrowska; Z.: Mesenchymal Chondro sarcoma. Arch. 88: 85, 1972.
[Figure - 1], [Figure - 2], [Figure - 3]