|Year : 1975 | Volume
| Issue : 3 | Page : 1-5
Orbital mesenchymal tumours (clinico-pathological aspects)
K Nath, R Gogi
J.N. Medical College, Aligarh, India
M. U. Institute, of Ophthalmology J.N. Medical College, Aligarh
|How to cite this article:|
Nath K, Gogi R. Orbital mesenchymal tumours (clinico-pathological aspects). Indian J Ophthalmol 1975;23:1-5
|How to cite this URL:|
Nath K, Gogi R. Orbital mesenchymal tumours (clinico-pathological aspects). Indian J Ophthalmol [serial online] 1975 [cited 2013 Jun 20];23:1-5. Available from: http://www.ijo.in/text.asp?1975/23/3/1/31317
The definitive benign mesenchymal tumours of the orbit are rare. This includes adipose, fibrous tissues, myomatous, cartilaginous and osseous tumours ,, .
Many of the lipomas reported in the literature are not tumours, for the large amount of normal fat in the orbit gives rise to diagnostic difficulties  . Pure fibroma of the orbit is a great rarity ,,, . Amongst 877 orbital tumours Reese  reported three such cases, and Mortada reported only two cases in a series of 900 orbital tumours  . This tumour appears usually before the age of 16 aid rarely after 30 years  . No specific sex preponderance is reported, It usually arises from the upper part of the periorbita , , but can also arise in the muscle cone  . Occasionally the tumour may be attached to the sheath of one of the rectus muscles , Fibroma arising from the optic nerve sheath has been described by Forrest  . He reported two cases of pure fibromas and another two of fibrolipoma.
Fibrosarcoma is also considered to be a rare tumour , . Forrest  came across only two cases in a series of 222 orbital tumours, and Silva  reported four such cases. Recently few more cases have been reported. ,,
Rhabdomyosarcoma was first described by Weber  in 1854, and other reports soon followed in the literature thereafter. However, it was in 1946 that Stout  published the first definite work on rhabdomyosarcoma. Five cases of orbital rhabdomyosarcoma were first described by Calhoun and Reese  . The whole subject was put on a firm footing by Porterfield and Zimmerman  and Jones, Reese and Kraul subsequently  .
Rhabdomyosarcoma is by far the commonest mesenchymal malignant tumour of the orbit amongst children [,11],,,, . The tumour has been reported to occur at an early age of one year and also at the age of two years ,.
Most authors have reported that the tumour is more common amongst males ,,,, , while there are others who find that there is no preponderance of either sex , .
Clinically it presents as a rapidly growing soft and fleshy tumour leading to an ever increasing proptosis in a child. The tumour has also been reported to arise following radiation therapy for retinoblastoma , . Porterfield and Zimmerman divided rhabdomyosarcomas into three types depending upon the microscopic appearance, i.e. embryonal, differentiated and alveolar types  .
| Material and Methods|| |
In the present study of 117 consecutive cases of primary orbital tumours, 13 mesenchymal tumours were diagnosed histopathologically. One case each of lieomyoma and rhabdomyosarcoma have already been reported , . The clinicopathological study of the other eleven cases is being presented here.
| Observations|| |
11 cases of mesenchymal tumours were diagnosed histopathologically, 6 were benign and 5 malignant. These tumours were seen in the patients between the age of 2½ to 60 years and affected 4 males and 7 females.
One case of orbital lipoma occured in a female aged 21 years. who presented with a mass projecting near the lateral canthus for eight years. There was limitation of lateral rectus of the affected side. Tumour mass was taken out through anterior orbitotomy.
The tumour was a yellowish lobulated and encapsulated softish mass, measuring 2.0 x 1.0 x 1.0 cm. Microscopically it showed the presence of capsule made up of fibrocollagenous tissue. The underlying tissue consisted of lobules of fat cells separated by fibrous septa containing a few thin walled blood vessels [Figure - 1].
Three cases of pure fibromas and two of fibrolipomas were seen. Clinically there was unilateral proptosis of gradual onset. One case occured during the first decade and two cases each in the second and fifth decades [Figure - 2]. In one case the tumour was removed by anterior orbitotomy and in other four through lateral.
The fibromas consisted of capsulated greyish white mass measuring 2.0 x 1.0 x 0.5 cm [Figure - 3] to 2.0 x 1.5 x 1.0 cm firm in consistency showing glistening white fibres on the cut surface. In fibrolipornas the cut surface showed yellowish soft areas in between the white fibres. Under the microscope there were interesecting bundles of fibres with fusiform cells [Figure - 4]; whereas in fibrolimpomas, there were groups of adult fat cells in between the fibres and fusiform cells [Figure - 5].
Of the three cases of fibrosarcoma, one patient was eight years and other two were nineteen years of age. Clinically there was rapidly increasing unilateral protusion of the eye of one to three months duration. In all the three cases tumour was diffusely infilterating and excenteration was done.
An irregular tumour masses measuring 4.0x3.5x2.5 cm to 4.5X4.0x3.0 cm were seen to surround the optic nerve and were infilterating the ocular muscles. There were fusiform and spindle shaped cells arranged in haphazard manner [Figure - 6]. The stroma was scanty. The cells had large nucleus with prominent coarse chromatin material and scanty cytoplasm. In many cells the mitotic figures both normal and abnormal were present [Figure - 7].
Two cases of orbital rhabdomyosarcoma in female children aged two and three years were seen. It presented a fungating mass protruding out of the orbit for four months. In one case excenteration was done whereas the other case left the hospital after the biopsy had been taken.
The excentrated mass from the orbit measured 4.5 x 4.0 x 3.5 cm. It was firm in consistency. The microscopic section showed a predominent picture of alveolar arrangement of neoplastic cells with loose fibroconnective septa in between. Most of the cells were round with hyperchromatic nuclei and eosinophilic cytoplasmic processes extending into the connective tissue septa [Figure - 8]. Some of the cells were freely floating into the alveolar spaces, while occasionally some tadpole shaped or multinucleated giant cells were also seen.
| Discussion|| |
Eleven cases of mesenchymal tumours included fibromas (five cases), fibrosarcoma (three cases), rhabdomyosarcoma (two cases) and lipoma (one case).
Lipoma is one of the rare tumours reported in the literature  . Many lipomatas reported in the literature are not tumours at all. A true lipoma must be encapsulated and there should be adult fat cells separated by fibrous septa  . This case of lipoma in this series fulfils the criteria of a true lipoma.
Fibromas usually appear before the age of 1 6 and rarely after 30 years  . Three cases in this study are under 30 while the other two are aged 50 and 60 years. All the tumours are well encapsulated and present the characteristic histopathological appearance. Cases of pure fibromas have been reported by other workers also ,,, . Forrest described two cases of fibrolipomas.
Three cases of fibrosarcomas have been recorded. It is one of the rarest orbital tumours  , although comparatively a high incidence (7.49%) has also been reported by Dass  , who recorded eight cases in a series of 108 primary orbital tumours. Fibrosarcomas of the orbit are comparatively benign and are rare after the age of 10 years  .
Rhabdomyosarcoma is considered to be the commonest of all the mesenchymal tumours of the orbit  but we could find only two such cases. This may be due to nonreference of most of the cases to our centre because of nonavailability of radio-therapy.
Formerly tumours were usually called rhabdomyosarcomas only when they arose at the site of striated muscles but it is now known that these tumours appear at other sites of the body including bladder, uterus, male genitalia and vagina. To explain this fact it was thought that the majority of them arose in embryonal tissue either from immature muscle tissue or from mesenchymal tissue with the potency for aberrant differentiation of muscle fibres , .
| Summary|| |
From a study of 117 cases of primary orbital tumours, 11 cases of mesenchymal tumours have been reported. Clinico-pathological aspect of these tumours has been described. Histopathological analysis shows, three fibroma, two fibrolipoma, one lipoma, three fibrosarcoma and two cases of rhabadomyosarcomas.
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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]