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| ARTICLE |
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| Year : 1966 | Volume
: 14
| Issue : 6 | Page : 239-245 |
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Orbital endothelioma
ST Puttanna1, U Ramchandraian2
1 Dept. of Ophthalmology, Minto Ophthalmic Hospital, Bangalore, India
2 Dept. of Pathology, Medical College, Bellari, India
| Date of Web Publication | 17-Jan-2008 |
Correspondence Address:
S T Puttanna
Dept. of Ophthalmology, Minto Ophthalmic Hospital, Bangalore
India
 Source of Support: None, Conflict of Interest: None  | Check |
How to cite this article:
Puttanna S T, Ramchandraian U. Orbital endothelioma. Indian J Ophthalmol 1966;14:239-45 |
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 | |  |
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 | | |
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 | | |
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 | | |
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 | | |
| 1. | Ali Mortada (1961) Arch. Ophth. 65: 636.  |
| 2. | Beitti (1915) A'Augenheilk, 54: 462. |
| 3. | Birch Hirsehfeld (1915) Arch. Ophth. |
| 4. | Eagleton. W.P. (1935) Arch. Ophth. 14: 1 |
| 5. | Forrest, A. W. (1949) Arch. Ophth. 41: 198. |
| 6. | Globes, J. H. (1937) Arch. Neurol. & Psychiat. 38: 667-712. |
| 7. | Hachim, H. (1949) Altrincham. England Vol. 3. John Schere alt & Sons. |
| 8. | Iliff, C. E. (1957) Trans. Amer. Ophthal. Soc. 55: 505. |
| 9. | Mallory, F. B. (1920) J. I. tiled. Res. 41. 349. |
| 10. | Musial. A. (1930) A. Ein, Fall Von. Endotheliaon des Augenlides 2. Agenteilk 71: 347. |
| 11. | Neame. H. (1923) Brit. J. Ophth. 7, 202 |
| 12. | Paton, L. (1905) Trans. Ophthal. Soc. U.K. 25: 240. |
| 13. | Puscarin, E. (1937) Arch. Ophth. (Paris) 1: 961. |
| 14. | Reese, A. B. (1941) Amer. J. Ophth. 24: 386. |
| 15. | -- (1951) "Tumours of the Eye", London. P. 411. |
| 16. | Stallard (1936) Brit. J. Ophth. 19: 576. |
| 17. | Willaimson Noble, F. A. (1923) Brit. J. Ophth. 7: 222. |
[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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