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ARTICLES
Year : 1975  |  Volume : 23  |  Issue : 3  |  Page : 1-5

Orbital mesenchymal tumours (clinico-pathological aspects)


J.N. Medical College, Aligarh, India

Correspondence Address:
K Nath
M. U. Institute, of Ophthalmology J.N. Medical College, Aligarh
India
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Source of Support: None, Conflict of Interest: None


PMID: 1236307

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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 2020 Feb 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 [7],[13],[24] .

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 [7] . Pure fibroma of the orbit is a great rarity [7],[13],[17],[24] . Amongst 877 orbital tumours Reese [24] reported three such cases, and Mortada reported only two cases in a series of 900 orbital tumours [17] . This tumour appears usually before the age of 16 aid rarely after 30 years [7] . No specific sex preponderance is reported, It usually arises from the upper part of the periorbita [12],[29] , but can also arise in the muscle cone [10] . Occasionally the tumour may be attached to the sheath of one of the rectus muscles [23],[25] Fibroma arising from the optic nerve sheath has been described by Forrest [8] . He reported two cases of pure fibromas and another two of fibrolipoma.

Fibrosarcoma is also considered to be a rare tumour [13],[24] . Forrest [8] came across only two cases in a series of 222 orbital tumours, and Silva [27] reported four such cases. Recently few more cases have been reported. [26],[31],[34]

Rhabdomyosarcoma was first described by Weber [32] in 1854, and other reports soon follow­ed in the literature thereafter. However, it was in 1946 that Stout [30] published the first definite work on rhabdomyosarcoma. Five cases of orbital rhabdomyosarcoma were first described by Calhoun and Reese [5] . The whole subject was put on a firm footing by Porter­field and Zimmerman [22] and Jones, Reese and Kraul subsequently [5] .

Rhabdomyosarcoma is by far the com­monest mesenchymal malignant tumour of the orbit amongst children [3][,11],[15],[21],[22],[24] . The tumour has been reported to occur at an early age of one year and also at the age of two years [16],[28].

Most authors have reported that the tumour is more common amongst males [4],[13],[14],[15],[24] , while there are others who find that there is no preponderance of either sex [11],[20] .

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 [10],[15] . Porterfield and Zimmerman divided rhabdomyosarcomas into three types depending upon the microscopic appearance, i.e. embryonal, differentiated and alveolar types [22] .


  Material and Methods Top


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 [18],[19] . The clinicopathological study of the other eleven cases is being presented here.


  Observations Top


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.

1. Lipoma

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].

2. Fibroma

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].

3. Fibrosarcoma

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].

4. Rhabdomyosarcema

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 Top


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 [24] . 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 [15] . 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 [7] . 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 [6],[8],[17],[24] . Forrest described two cases of fibrolipomas.

Three cases of fibrosarcomas have been re­corded. It is one of the rarest orbital tumours [13] , although comparatively a high incidence (7.49%) has also been reported by Dass [6] , 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 [7] .

Rhabdomyosarcoma is considered to be the commonest of all the mesenchymal tumours of the orbit [35] 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 [2],[33] .


  Summary Top


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.

 
  References Top

1.
Apte, P.V., Patil, R.V. and Lemade, P.T. 1971, Orient. Arch. Ophthal., 9, 220.  Back to cited text no. 1
    
2.
Ashton, N. 1958, Trans. Ophthal. Soc., U.K., 78, 83.  Back to cited text no. 2
    
3.
Ashton, N. and Morgan, G. 1965, Jour. Clin. Path., 18, 699. Quoted, Kirk, R.C., and Zimmerman, L.E. (1969), Arch. Ophthal., 81, 559.  Back to cited text no. 3
    
4.
E. Blaxter, P.L. and Lindley-Smith, J. 1958, Trans. Ophthal. Soc., U.K., 78, 83.  Back to cited text no. 4
    
5.
Calhoun, F.P. Jr., and Reese, A.B. 1942, Arch. Ophthal., 27, 558.  Back to cited text no. 5
    
6.
Dass, S.P. 1963, Jour. All India Ophthal. Soc., 11, 38.  Back to cited text no. 6
    
7.
Duke Elder, S. 1952, : "Text-Book of Ophthalmology", 5, P. 5532, & 5596. Kimpton, London.   Back to cited text no. 7
    
8.
Forrest, A.W. 1949, Arch. Ophthal., 41, 198.   Back to cited text no. 8
    
9.
Forrest, A.W. 1962, Int. Ophthal. Clin., 2, 543.   Back to cited text no. 9
    
10.
Fowler, J.G. and Terplan, K.L. 1942, Arch. Ophthal., 28, 263.  Back to cited text no. 10
    
11.
Frayer, W.C. and Enterline, H.T. 1959, Arch. Ophtal , 62, 203.  Back to cited text no. 11
    
12.
Garcia Miranda, A. 1947, Ophthalmologica, 113, 149.  Back to cited text no. 12
    
13.
Hogan, M.J. and Zimmerman, L.E. 1962, "Ophthalmic Pathology, An atlas and textbook", 2nd Ed. P. 747, Saunders, Philadelphia.  Back to cited text no. 13
    
14.
Horn, R.C. Jr., and Enterline, H.T. 1958, Cancer, 11, 181.  Back to cited text no. 14
    
15.
Jones, I.S., Reese, A.B. and Kraut, J. 1966, Amer. J. Ophthal, 61, 721.  Back to cited text no. 15
    
16.
Lederman, M. 1956, Brit. J. Ophthal., 40, 592.   Back to cited text no. 16
    
17.
Mortada, A. 1971, Brit. J. Ophthal., 55, 350.  Back to cited text no. 17
    
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21.
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22.
Poterfield, J.F. and Zimmerman, L.E. 1962, Virchows Arch. Path., Anat., 335 : 329. Quoted Jones, I.S., Reese, A.B., and Kraut, J. (1966).   Back to cited text no. 22
    
23.
Reese, A.B. 1941, Amer. J. Ophthal., 24, 386.  Back to cited text no. 23
    
24.
Reese, A.B. 1963, "Tumors of the eye"., 2nd Ed., Pp. 441, 455, 458, 535. Harper and Row, N.Y.  Back to cited text no. 24
    
25.
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27.
Silva, D. 1968, Amer. J. Ophthal., 65, 318.  Back to cited text no. 27
    
28.
Sood, G.C., Sen, D.K., Diwan, R. and Aurora, A.L. 1970, Brit. J. Ophthal., 54, 203.  Back to cited text no. 28
    
29.
Stockes, W.H. and Bowers, W.F. 1934, Arch. Ophthal., 11, 279.  Back to cited text no. 29
    
30.
Stout, A.P. 1946, Ann. Surg., 231, 447.  Back to cited text no. 30
    
31.
Templeton, A.C.1971, Brit. J. Ophthal., 55, 254.  Back to cited text no. 31
    
32.
Weber, 1854, cited by Jones, I.S., Reese, A.B., and Kraut, J. (1966).  Back to cited text no. 32
    
33.
Willis, R.A. 1948, "Pathology of Tumours". P. 757, C.V. Mosby Co., St. Louis, Quoted, Reese, A.B. (1963).  Back to cited text no. 33
    
34.
Yonoff and Schief 1966, Cancer, 19, 1711,  Back to cited text no. 34
    
35.
Zimmerman, L.E. 1967, Arch. Ophthal:, 78, 166.  Back to cited text no. 35
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]



 

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