Ocular and orbital tumours and tumour-like lesions - a clinico-pathological study of 64 casesRB Deshpande, Leena P Deodhar
Department of Pathology & Microbiology, Seth G. S. Medical College, Parel, Bombay-400 012., India
Sixty-four ocular and orbital tumours were encountered during a period of six years, in the department of Pathology and Microbiology, Seth G.S. Medical College, Bombay, of which nineteen were ocular and forty-five were orbital tumours. Retinoblastoma was the commonest tumour amongst the first series while vascular tumours formed the largest group amongst the orbital tumours. The series also included rare tumours like orbital rhabdomyosarcoma, chloroma and diktyoma. Details about clinical presentation and gross and microscopic findings of these tumours are presented.
Tumours arising from the eyeball (ocular tumours) and those arising from other structures found in the orbit like fat, skeletal muscle, nerves, blood vessels, lacrimal glands, lymphoid follicles etc. which are labelled as orbital tumours can be primary or metastatic. Besides these, the malignant tumours from the contiguous anatomic areas such as nasal cavity, paranasal sinus, parotid glands, skin of the forehead, eyelid can also invade the orbital space. Thus, the variety of tumours that can originate, grow, invade and lodge in the relatively small orbital cavity is amazing. A few case reports on rare tumours as well as a review of 100 cases of retinoblastoma are available in Indian literature. The present paper deals with common as well as rare ophthalmic tumours.
During a period of 6 years, there were 64 specimens of orbital and ocular tumours available for study. 62 were surgical biopsies while in 2 cases postmortem material was available. Details regarding clinical history, investigations, surgical procedure employed and follow up notes of all cases were recorded.
For the sake of convenience, the ocular and orbital tumours are discussed separately. Ocular tumours included 5 cases of epithelial tumours of conjunctiva and cornea, one case of diktyoma, 12 cases of retinoblastoma and one case of melanoma of the uveal tract. All the 5 cases of epithelial tumours of conjunctiva and cornea were seen in elderly patients and microscopically showed features of well differentiated squamous cell carcinoma.
A solitary case of diktyoma was seen in a girl aged 5 years who came with loss of vision, proptosis and yellow reflex. Clinically, she was diagnosed as a case of retinoblastoma and enucleation was performed. Cut section of the eyeball showed a soft greyish white friable tumour mass in the anterior part with no extraocular extension. Optic nerve was free from the growth.
Microscopically: Tumour consisted of bands of cells arranged in one or several rows forming intricate convolution upon folded membranes. At places tumour cells formed rosettes and cribriform pattern See [Figure 1] on page 88A. No pigment cells or cartilage was seen in any of the section.
Embryonal medulloepithelioma is a rare malignant tumour of the eye arising from the unpigmented epithelium of the ciliary body. Only four cases have been reported from India. ,,, Diktyoma is a slow growing tumour, and although related to retinoblastoma, it has a better prognosis.
Retinoblastoma: There were 12 cases in the age group 2 to 5 yearss. In two cases, the tumour was bilateral. Most of the patients presented with proptosis, loss of vision and yellow reflex. In two cases; post-mortem material was available where following the enucleation of the original tumour mass in the eye, the child developed meningeal seedlings and also greyish white firm tumour nodules on the base of the brain.
In 10 cases, the cut section of the eye revealed greyish white, soft friable tumour mass in the globe. No definite site of origin could be made out. In twc cases the tumour showed extension into the orbit and the optic nerve. See [Figure 2] on page 88A.
Microscopically: The tumour consisted of large round or oval cells with hyperchromatic nuclei. In one case typical Flexner-Wintersteiner rosettes were seen; here, the tumour cells were arranged like the spokes of a wheel in a palisade form round a central cavity containing some eosinophilic material See [Figure 3] on page 88A. Remaining cases showed large areas of necrosis, haemorrhage amidst which islands of tumour cells were seen. Calcification was noted in two cases. Microscopic invasion of the optic nerve was seen in six cases. Retinoblastoma is the most common intraocular tumour of childhood; and its frequency varies from 1:34,000 to 1:14,000 according to Francois.' In the present series the average age incidence was 2.8 years at the time of diagnosis while Shrikhande and Sirsat  have reported the same as 3.9 years.
Two forms of retinoblastoma are described, a herditary one and sporadic one-the second being more common. The percentage of bilateral involvement is significantly higher in familial than in sporadic cases. In this series, bilateral tumours occurred in 8.6% cases.
Retinoblastoma can spread by direct extension into the orbit or via lymphatics or blood stream. Extension into the central nervous system is through the optic nerves.
A single case of malignant melanoma of the uveal tract was encountered in a 60 year old female who came with glaucoma and diminished vision of 6 months' duration. The eyeball was enucleated and it showed a brown, soft tumour mass occupying the globe. Microscopically, the tumour mass consisted of spindle shaped cells arranged in sheets with large amount of melanin pigment in the cytoplasm. The patient expired 11 years later. No autopsy examination was available.
Orbital tumours: [Table 1] shows different types of orbital tumours encountered in this study.
There were 9 cases of haemangioma. The average age was 20 years. The tumours were soft, cystic, haemorrhagic masses and microscopically showed features of capillary haemangioma in 2 cases and cavernous haemangioma in 7 cases. Hemangiomas are said to be the most common tumours of the orbit,  the frequency varying from 8 to 16.2%.  The tumours usually appear in children and younger age group and they are in close association wih ocular muscles. Surgical excision of the tumour is the best method of treatment.
Eight cases of orbital cysts-6 dermoid cysts and 2 epidermoid cysts were noted in this series. Henderson  (Mayo Cliniq series) has reported an incidence of 2.8%. Like hemangioma, surgical removal of the cysts is reported to give a complete cure.
There were solitary cases of myxoma, osteod ostoma, lipoma, neurilemmoma and rhebdomyosarcoma, Out of these, last case needs a special mention. The patients was a five year old girl who came with propotosis and restriction of eyeball movements. X-rays showed a soft tumour mass pushing eyeball forward. The tumour was adherent to the eyeball, irregular, firm and greyish white in colour.
Microscopically: The tumour showed large, rounded pleomorphic cells with hyperchromatic nuclei. There were many racket shaped cells, strap cells and giant cells. Some of the strap cells showed cross striations on PTAH staining. Rhabdomyosarcomas are among the more common malignant tumours of the soft parts, and they form a complex group of tumours with various adult and embryonal forms.  Of the 62 primary rhabdomyosarcomas of the orbit reviewed by Jones,  60 were in children aged below 16 years. In Mayo Clinic Series  there were 10 cases out of a total of 465 orbital tumours. A great majority of juvenile rhabdomyosarcomas melastatise freely and widely, more commonly through the blood stream, but sometimes through the lymphatic vessels.  The patient in this series has been followed up for last 6 months with no evidence of metastasis or recurrence.
Malignant lymphoma: There were 8 cases of malignant lymphoma in the present study. There was no generalised lymphadenopathy in any of these cases. Blood examination revealed normal values. Gross appearance of the tumour was that of greyish white, soft mass with homogenous appearance on cut section. Microscopically, there were 5 cases of well to moderately differentiated lymphocytic lymphoma while 3 were reticulum cell sarcoma (histiocytic lymphoma); reticulin stain showed large amount of reticulin network. The incidence of malignant lymphoma in the orbit varies from 7.4%  to 11.8%  In general, primary orbital lymphomas are said to have a better prognosis than systemic lymphoma. 
Chloroma: A single case of chloroma in a girl aged 8 years was seen in this series. The patient was a known case of acute myeloblastic leukemia who came with proptosis, restriction of the eyeball movement and pain in left eye of 25 days' duration. The blood and the bone marrow examination revealed a large number of myeloblasts and total white cell count was 40,000/cmm. Grossly the tumour was soft, irregular and slightly greenish in colour. Cut section showed a similar appearance.
Microscopically: The tumour showed a large number of immature blast cells with large round hyperchromatic nuclei. Reticulin stain did not show any reticulin fibres.
The distinctive feature of this tumour is its greenish colour due to myeloperoxidase; this is responsible for its name.  In its clinical behaviour chloroma resembles other leukemias except that it has a greater propensity to involve the orbit. It is seen more often in males and the tumour deposits frequently involve both orbits before the disease terminates fatally.  The present patient expired 2 months after the removal of the tumour.
There were solitary cases of mixed tumour of the lacrimal gland and a secondary tumour infiltrating into the orbit, the primary being carcinoma of the ethmoid.
Meningiomas: There were 7 cases. Patients presented with proptosis and pain in the eye. The tumour was mainly located in posterior part of the orbit. In 3 cases, no recurrence was seen after removal of the tumour. No follow up was available in remaining 4 cases.
Gross: In all the cases, tumour mass was greyish white in colour and firm.
Microscopically: Of the 7 cases studied, 4 were psammomatous meningiomas, 2 syncitial and 1 fibroblastic.
Meningiomas are known to arise from the optic nerve sheath or from ectopic rests of arachnoid cells in the orbit.  Factors such as position of the neoplasm, its size, manner of its growth and its proximity to important structure in the orbitinfluence the prognosis. , Prognosis is said to be better in patients with meningioma primary in the orbit rather than in those which are secondary to intracranial meningioma. 
Inflammatory pseudotumours: There were 6 cases of orbital inflammatory pseudotumours. Patients presented primarily with proptosis and pain in the eye; following excision of the mass, 3 patients who were followed up showed no recurrence for 2½ years. The remaining 3 patients were not available for follow up. In all cases, small biopsy tissues-firm, grayish white-were available for study.
Microscopically: Sheets of mature lymphocytes infiltrating the, fibrous connective tissue were seen. There were areas showing plasma cells, neutrophils and also a few germinal centres.
The term pseudotumour is used when surgical exploration of the orbit fails to reveal any evidence of a mass in a patient who clinically is supposed to have an orbital tumour. In Mayo Clinic Series  there were 37 patients of the total 465 tumours giving a percentage of 8, while incidence in the present series was 9.2 per cent.
Collectively, these pseudotumours constitute a large and important segment of orbital tumour problems in adults because of relative frequency, obscure etiology and ineffectual therapy. The treatment for inflammatory pseudotumours is mainly surgical while some believe in steroid therapy and radiotherapy. 
We are grateful to the Dean, Seth G.S. Medical College and K.E.M. Hospital, Bombay for allowing us to use the hospital material. We are also grateful to Dr. A, P. Desai, M.D., Department of Neuropathology for allowing us to use the material from his department. We thank the Head of the Department of Ophthalmology for referring the cases.
[Figure 1], [Figure 2], [Figure 3]