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   Table of Contents      
ARTICLE
Year : 1966  |  Volume : 14  |  Issue : 6  |  Page : 239-245

Orbital endothelioma


1 Dept. of Ophthalmology, Minto Ophthalmic Hospital, Bangalore, India
2 Dept. of Pathology, Medical College, Bellari, India

Date of Web Publication17-Jan-2008

Correspondence Address:
S T Puttanna
Dept. of Ophthalmology, Minto Ophthalmic Hospital, Bangalore
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Puttanna S T, Ramchandraian U. Orbital endothelioma. Indian J Ophthalmol 1966;14:239-45

How to cite this URL:
Puttanna S T, Ramchandraian U. Orbital endothelioma. Indian J Ophthalmol [serial online] 1966 [cited 2020 Dec 2];14:239-45. Available from: https://www.ijo.in/text.asp?1966/14/6/239/38664

Primary orbital tumours in general and orbital endothelioma in particular are rare. These rare but important tu­mours by virtue of their anatomic po­sition and of their local malignancy act as sites for disease within the orbit on the one hand and disease within the cranium on the other. Because of this fact, it is not surprising that there is in­terdependence between Ophthalmolo­gists, Neuro-Surgeons and Pathologists with regard to certain problems con­cerning these tumours. Our cases are amply illustrative as they presented great difficulty in diagnosis because of the atypical clinical picture and his­tologic structure. These cases were diagnosed as fibrosarcoma of the lid, anaplastic carcinoma of the lacrymal gland and orbital endothelioma by different pathologists.


  Case Report Top


Case 1 : S. a Hindu male, aged about 6 years, was admitted into the ophthal­mic department of Krishnarajendra Hospital, Mysore, on 13-7-1961, for swelling of the upper lid of the right eye of 3 months duration. Previously he had a small swelling in the same region and was operated on 13-3-1961. It was of 6 months duration and the biopsy examination was not done then. There was no history of trauma, syphi­litic infections, tuberculosis or fever.

Present History: Since the growth recurred rapidly causing discomfort, pain and proptosis of the right eye­ball, the boy saught admission into this hospital again. The growth was increasing rapidly since 3 months and the vision was failing gradually.

On examination, there was chemosis of the conjunctiva and intense oedema of the lids. The right eye was prop­tosed and the globe displaced forwards and downwards. The ocular move­ments were limited in all directions due to the large size of the growth. The mass of growth measured 7.5 cms x 3.5 cms in size. It was palpable under the supra-orbital margin and ex­tended to the outer orbital wall push­ing the globe inwards. The swelling was tender on pressure, firm in consist­ency and fleshy in appearance with multiple points of bleeding. The tumour appeared to arise from the upper part of the globe and the mass projected outwards 8 m.m. beyond the supra-orbital margin (Plates l and la.) The cornea and pupil were normal un­til ulceration and perforation of the cornea with prolapse of iris took place five days before the operation. The vision at the time of admission was R.E. counting fingers from 3 m. and L.E. 6/12.

Biopsy was done on 18-7-1961 and it was reported as fibrosarcoma on 20-7-1961.

E.N.T. examination : Nothing ab­normal was detected.

Radiograms of skull, chest and long bones: No abnormality detected.

Wassermann's Reaction was nega­tive.

R.B.C.'s 3.60 m/c.mm., W.B.C.'s 7200/c.mm.

Hcxmoglobin 62%

Polymorphs 60%, Eosinophils 10%, Lymphocytes 30%.

The condition was clinically diag­nosed as fibrosarcoma of the upper lid and as the globe was perforated, re­moval of the neoplastic tissue with the globe was decided. He was operated on 25-7-1961. An incision was made along the upper lid margin throughout its extent. The orbicularis muscle fibres were separated. The neoplastic tissue bulged out of the lid incision. It appeared encapsulated and it was removed by blunt dissection along with the globe. The wound was su­tured with drianage tube in place. The post-operative period was un­eventful and the patient was discharged from the hospital on 2-8-1961.

A course of deep X-Ray therapy: (2500 r) was given from 18-9-61 to 30-9-61; -- 7 x 7 cm. field, F.S.D. 50 Cms. at 200 k.v.: filter 1 mm. copper.

Pathological appearance and report: The tumour measured 7.5 x 5 x 5 cms, and appeared as an oval mass attached to the supero-lateral aspect of the globe extending as far as the optic nerve pos­teriorly [Figure - 2]. The eyeball was com­pressed by the neoplastic tissue [Figure - 3]. Cut surface appeared whitish grey in colour, homogeneous and encapsulated. The eye ball was free except at one point at which it was adherent at the sclera. Optic nerve was free and nor­mal.

The histological picture varied con­siderably. The neoplastic cells were arranged in sheets around blood ves­sels, with abundant intercellular ground substance. The cells appeared atypical showing metaplastic variations in places to spindle cells resembling those of fibrosarcoma [Figure - 4]. At other places the cells were crowded around blood vessels giving an appearance of peri­thelioma [Figure - 5]. The intercellular stroma had minimal amount of colla­gen tissues as shown by Van Gieson and Mallory stains. An intimate rela­tion of reticulin fibres to individual neoplastic cells was revealed by reti­culin stain [Figure - 6]. The tumour cells were oval or spindle shaped with gra­nular blue staining cytoplasm and hyperchromatic nucleus with well de­fined chromatin network and distinct nucleolus. Mitotic figures were also frequently seen. Cell vacuolation was conspicuous [Figure - 7] and intercellular spaces which appeared like clefts were infrequent in the section. In addition, mucoid-degeneration was also noted amidst the tumour cell mass [Figure - 8]. The lacrymal gland was pushed to one side and it presented normal histologi­cal appearance though there were signs of surface infiltration by the tumour cells. The muscle cones and nerves could not be identified. From the gross and microscopic appearances, a diag­nosis of endothelioma of the orbit was made.

Case 2: K, a Hindu male, aged about 9 years, was admitted to the ophthalmic department of Krishna­rajendra Hospital, Mysore, on 27-11-1961, for swelling of the upper lid and protrusion of the left eye, of one month's duration.

Previous history : The patient suf­fered from earache for five days, one month back and since then noticed pain and redness in the left eye with forward protrusion of the left eye for which he was admitted at Mercara Hospital and was treated for 10 days.

Family history : He is the 4th mem­ber of the family and has one sister, aged 18 years, who has nothing ab­normal in her.

Present history : Since the growth in­creased rapidly causing discomfort pain and lacrimation and the cornea showed ulceration in the centre, he was referred to our department for further investigations and treatment.

On examination, there was intense chemosis of the conjunctiva and oedema of both lids with prominent veins and there was marked proptosis of the left eve. The globe was displaced for­wards and downwards and the whole cornea ulcerated. The ocular move­ments were limited in all directions. There was a firm mass 5.0 x 2.5 cms, felt along the outer orbital margin pushing the globe downwards and slightly inwards. The swelling was very tender on pressure. The tumour appeared to arise from the upper part of the globe and the mass projected outwards to 10 mm., beyond the supra­orbital margin.

Nothing abnormal was detected in the examination of the Ear, Nose and Throat.

Radiogram of the skull, chest and long bones : No abnormality.

Wassermann Reaction was negative.

The condition was clinically diag­nosed as an orbital neoplasm and re­moval of the tumour with the globe was decided. Exenteration of the or­bit, saving the lids, was done on 9-12-1961. The swelling appeared en­capsulated and the neoplastic tissue surrounded the optic nerve as well as infiltrated into the lacrimal gland. [Figure - 9]. The optic nerve appeared normal.

The Section showed sheets of vacuo­lated cells with slender vascular spaces without perithelial arrangement. Reti­culin stain shows argyrophilic fibres and mature collagen fibres [Figure - 10],[Figure - 11],[Figure - 12].


  Discussion Top


Endotheliomata of the orbit are rare. At the New York Eye and Ear Infirmary in 1930, there were only 9 cases recorded as having orbital tu­mours out of a total number of 35,869 patients. At the Royal Ophthalmic Hospital, London, in 1930, with 52,372 patients, only one case was admitted to the hospital with the diagnosis of orbital neoplasm. Birch-Hirschefeld (1915) found 4 cases among 200,000 patients at Leipzig. Of 222 intra-orbi­tal tumours reviewed by Forrest, there were 88 primary tumours which in­cluded one case of angio-endothelioma. Iliff (1957) found three cases. Reese encountered 6 angioblastic haeman­gioma and 1 haemangioendothelioma in a series of 44 orbital tumours. Aly Mortada (1961) found 4 endothelioma among 120 histologically diagnosed primary orbital tumours. These tu­mours might arise from the lids or lacrymal sac. Musial (1930) reported a case of endothelioma invading the orbit from the lid. Similarly, Bietti (1915) reported endothelioma infiltrat­ing into the orbit from the lacrymal sac. Till date, these are the first two cases of primary orbital endothelioma recorded in our hospital statistics.

The varied histological picture and the indefinite histogenesis led to the use of many different terms to desig­nate this tumour. According to Reese, this group consisted of haemangioma, meningoma of the optic nerve sheath and mixed tumours of the lacrymal gland. Others described them as an­gioendotheliomata, endotheliosarco­mata and angiosarcomata. Based on their histology, they were often referred to as alveolar sarcomata or cylindro­mata-Puscarin. As these endothelial cells presented metaplastic variations they were designated as fibrosarcomata and fibroblastomata. Some called them peritheliomata on the presumption that the tumour cells arose from the adven­titial perithelium of the blood vessels. But, Paton, Williamson Noble and Eagleton, designated these tumours as endotheliomata.

The origin of endothelioma is still a controversial subject. Whether they are truly primary tumours which arise within the orbit or mere extension from similar growths within the cranium or other surrounding structures is not clear.

Hachim (1949) was of the opinion that the neoplastic cells were endothe­lial in nature and they lined the capil­laries. They passed through various stages. Mucoid degeneration in a group in a large mass of endothelial cells occurred resulting in irregular spaces surrounded by endothelial cells of the tumour. The cells projecting into the inner surface and into the lumen of the blood spaces were merely necrotic remnants of the endothelial cells formerly occupying the spaces. Gradually the spaces became lined by flat endothelial cells giving the ap­pearance of neoplastic capillary forma­tion. Occasionally red blood corpus­cles are also seen within the vascular spaces. Similar growths of lacrymal sac, lids, caruncle, nasal sinuses, periorbita arising from the angioblas­tic tissue in the walls of the blood vessels are encountered. Aly Mortada (1961) was of the opinion, that these tumours arose from embroyonic vas­cular endothelium.

Stallard (1935) on the other hand thought that the endothelial cells were derived either from those adjacent to the dura and lining the subdural space or from the cells covering the fine connective tissue and elastic tissue which formed the arachnoid matter. Mallory (1920) considered the term endothelioma as fundamentally incor­rect and suggested instead the term arachnoid fibroblastoma as more suit­able because the endothelial cells were merely differentiated connective tissue cells. This conception was supported by the presence of collagen and elastic fibrils in these tumours probably pro­duced by the endothelial cells under conditions of tumour formation. Neame (1923) also noted in these tu­mours more fibrous tissue than could arise by the permeation of the neoplas­tic tissue into the dural sheath and the sclera with subsequent expansion of the fibrous tissue layers and lamella; of those structures.

Globus (1937) was of the opinion, that the mesenchymal tissue filling the space between the skin and the neuro­ectoderm which was the primordium of all the brain coverings was the site of origin of these tumours. The undiffer­entiated meningeal primordium with its multi-potentiality could elaborate into bone, collagen tissue, endothelial cells and vascular channels. The amount of each varied with the stage of differentiation. If vascular destiny predominated the tumour assumed haemangiomatous character and an en­dotheliomatous appearance if endothe­lial cells dominated the picture. Thus, the histogenesis of these tumours has remained unsettled.

The histologic picture of these tum­ours is very complex and presents great difficulty in diagnosis as in our cases. Our cases were diagnosed as fibrosarcoma, neurofibrosarcoma, ana­plastic carcinoma of the lacrymal gland endothelioma and so on by different pathologists. The cell vacuo­lation noted in one case also suggested the diagnosis of liposarcoma [Figure - 6]. The possibiity of these tumours were excluded by special stain for fat, reti­culin, nerve and muscle tissue. The spindle cells were not the predominat­ing type in the section, nor were there sufficient collagenous fibres to prove its origin from the fibrous tissue of the orbit or periorbita. Further, periorbita was intact in our cases. From the histological presentation, it was impos­sible to diagnose the precise site of origin of these endothelial cells, whe­ther they were from the lining cells of dura, arachnoid matter, blood vessels or lymphatics as in the case presented.

Endotheliomata might exhibit slow growth and might recur though rarely. The history of our Case-l, in all pro­bability indicated a recurrence of the tumour in about 3 months.

These tumours are characterised by local malignancy with strong tendency to infiltrate resulting in destruction of surrounding tissues. The sclera in the vicinity may show evidence of infiltra­tion by tumour cells.

In some cases, there may be diffuse thickening of the adjacent bone. In our case, there was neither the scleral nor the periorbital infiltration by the neo­plastic cells. There was no thickening of the adjacent bone. In case 2, infil­tration of lacrymal gland tissue with neoplastic cells was seen.

These tumours may occur at any age but usually appear after the second decade of life. The ages of our cases were 6 and 9 years only.

Clinical manifestations vary with the size of the tumour and its rate of growth. Proptosis was a common and constant finding in many authors' series. This feature was noted in our cases with displacement downwards. The mechanical pressure on the globe resulted in corneal exposure, keratitis and ulceration with loss of vision. The loss of vision might result from optic atrophy, corneal ulceration, papille­dema, thrombosis of the central re­tinal vein or glaucoma probably caus­ed by pressure on the vertex system of veins. Pain was noted in our cases as a prominent symptom.

The excision of the globe with the tumour was resorted to, as there was ulceration of the cornea with perfora­tion of the globe and loss of vision.


  Summary Top


Two unusual cases of orbital endo­thelioma are presented.

Prevailing views regarding the nom­enclature and histogenesis of these tumours are reviewed.

Clinical and histopathological pecu­larities including diagnosis difficulties are discussed and illustrated.


  Acknowledgement Top


The technical assistance of Mr. Paul Jayaraj, Research Assistant, Central Food Technological Research Insti­tute is gratefully acknowledged. We are highly thankful to Dr. S. Sreerama­chari Neuro-Pathologist, All India Institute of Mental Health, for his valuable criticism and suggestion in the preparation of this paper.[17]

 
  References Top

1.
Ali Mortada (1961) Arch. Ophth. 65: 636.  Back to cited text no. 1
    
2.
Beitti (1915) A'Augenheilk, 54: 462.  Back to cited text no. 2
    
3.
Birch Hirsehfeld (1915) Arch. Ophth.  Back to cited text no. 3
    
4.
Eagleton. W.P. (1935) Arch. Ophth. 14: 1  Back to cited text no. 4
    
5.
Forrest, A. W. (1949) Arch. Ophth. 41: 198.  Back to cited text no. 5
    
6.
Globes, J. H. (1937) Arch. Neurol. & Psychiat. 38: 667-712.  Back to cited text no. 6
    
7.
Hachim, H. (1949) Altrincham. Eng­land Vol. 3. John Schere alt & Sons.  Back to cited text no. 7
    
8.
Iliff, C. E. (1957) Trans. Amer. Oph­thal. Soc. 55: 505.  Back to cited text no. 8
    
9.
Mallory, F. B. (1920) J. I. tiled. Res. 41. 349.  Back to cited text no. 9
    
10.
Musial. A. (1930) A. Ein, Fall Von. Endotheliaon des Augenlides 2. Agen­teilk 71: 347.  Back to cited text no. 10
    
11.
Neame. H. (1923) Brit. J. Ophth. 7, 202  Back to cited text no. 11
    
12.
Paton, L. (1905) Trans. Ophthal. Soc. U.K. 25: 240.  Back to cited text no. 12
    
13.
Puscarin, E. (1937) Arch. Ophth. (Pa­ris) 1: 961.  Back to cited text no. 13
    
14.
Reese, A. B. (1941) Amer. J. Ophth. 24: 386.  Back to cited text no. 14
    
15.
-- (1951) "Tumours of the Eye", London. P. 411.  Back to cited text no. 15
    
16.
Stallard (1936) Brit. J. Ophth. 19: 576.  Back to cited text no. 16
    
17.
Willaimson Noble, F. A. (1923) Brit. J. Ophth. 7: 222.  Back to cited text no. 17
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9], [Figure - 10], [Figure - 11], [Figure - 12]



 

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