Original Article

Orbital tumors may be primary, secondary, metastatic extension from adjacent tissues – sinuses, lids, Eye ball or manifestation of leukaemia. Tumors may be benign or malignant; in children 90% are benign (Cystic) and 10% malignant. Studies by various authors have given variable incidence of different tumors, depending on age, race, region and study period.

Lymphoid tumors, inflammatory lesions (Pseudo-tumor muccoele) vascular and cystic lesion (dermoid and epidermoid are most common¹. There are conditions, which mimic orbital tumors such as thyroid ophthalmopathy usual presentation is axial proptosis, decreased vision, restriction of E.O.M, pain, inflammation and cosmetic disfigurement. The initial clinical evaluation of patient with orbital mass (lesion is frequently inclusive to arrive at corrective diagnosis clinical examination, blood tests, X-Ray P.N.S and Rhinological examination, B-Scan ultrasound, CT Scan and MRI are done. Biopsy remains gold standard; ultrasonography is a good diagnostic tool especially in tumors of solid, cystic variety and thyroid orbitopathy².

Primary orbital tumors if not treated on time and adequately cause morbidity and mortality by local extension and systemic metastasis.

MATERIAL AND METHODS

This is a retrospective study includes all cases confirmed by histology. We excluded cases of thyroid ophthalmopathy, congenital bony anomalies, orbital varicose veins, pseudo tumors who responded to retrobulbar and systemic steroids. The cases where histopathological slides showed multi cellular and anaplastic cell appearance and diagnosis was speculative were also excluded.

We sought opinion of paediatrition, radiologist and clinical pathologist where necessary biopsy was done in all cases. Blood tests, CT Scan, MRI were done in selected cases to assess site, size, spread to surrounding structures and plan surgical approach.

CT Scan and MRI helped clinically and morpholo-gically to differentiate orbital infections from benign and malignant tumor of epithelial and connective tissue origin. Tumor within muscle cone or advanced tumors invading sinuses on anterior cranial fossa were referred to ENT and Neuro Surgeon respectively.

RESULTS

Age of study cases varied from 6 months to 80 years and gender wise male were 23 and female 19 (Table 1). The most common tumors were Lymphoma, squa-mous cell carcinoma and retinoblastoma comprising 6 of each (Table 2). While other tumors included Fibro Sarcoma, Lacrimal Gland Tumor, Socket Tumor, tuberculous granuloma, neurofibromatosis (3 each), optic nerve meningioma, Dermoid Cyst, Lymphoid Hyperplasia of Lacrimal Gland (2 each) and Schwa-nnoma, Metastatic Tumor, Porocarcinoma (1 each) (Table 2). In squamous cell carcinoma 04 cases were extension from limbus (Fig. 6) and 2 cases were direct spread from lid (Fig 7).

Regarding Fibrosarcoma, first case of two year old female child had grown to larger dimensions just within three months time. It shows rapid and aggressive growth in children¹. In a second case of 24 year old male, it had grown slowly and was well differentiated. In a third case 55 year old woman, it had grown slowly over a period of 5 years and was painless (Fig 3). On attempted exentration, it was massive growth which bled profusely and had eroded bony walls of maxillary sinus, lateral wall of nose and roof of orbital fossa. Patient died three weeks post-operatively due to concurrent infection. As regards lacrimal gland tumor adenoid cyst carcinoma occurred in a 40 year old female. This tumor had local infiltrative and metastatic potential. It responds to radio therapy but is not radio curable¹. Rest three tumors were one case epidermoid carcinoma of lacrimal gland origin and two cases of lymphoid hyperplasia.

Table 1: The gender breakup of cases

Table 2: The percentage of different types of tumors

Fig. 1: Fibrsarcoma

Fig. 2: Lymphoma H.E Staining

Fig. 3: Schwannoma H.E Staining

Fig. 4: Meningioma Optic Nerve

Fig. 5: Schawanoma

Fig. 6: Squamous Cell Carcinoma

Fig. 7: Squamous Cell Carcinoma Lid.

Two cases of optic nerve menigioma were recorded. In both patients enucleation was done: one 24 year old male had progressive proptosis, pain, lid oedema, chemosis (Figure 4), for such growths similar description has been described with age less than 10 years in literature¹¹. Regarding nerve sheath tumors, one schwannoma (Neurolimoma) and two Neurofibroma were recorded. Schwannoma has been reported in two studies^5,8 but without mention of histological pattern and duration of onset.

Our case (Fig 3) with 5 year duration is unique: bulky mass and easily enucleated out without blood loss contrary to our expectations. Microscopically both Antoni A-Spuidle cells in cords and whorls plus Antoni B-stellate cells with mucoid stroma, coexisted in some cross sectional view¹.

A nine year old boy presented with painless proptosis of right eye of recent onset. After clinical examination retrobulbar leukaemia deposits were suspected. Peripheral blood film revealed blast cells. Patient was referred to hematologist and was lost to follow up.

In 50 year old man with proptosis of few years duration exentration was done. A diagnosis of poro carcinoma was made by Histopathologist after examination of orbital contents. Incidentally secondary tumors with extension from adjacent sinuses, nose brain were not encountered in our collection of 24 years duration.

DISCUSSION

Incidence of Lymphoma was higher as reported in recent studies^3,4 followed by squamous cell carcinoma and Retinoblastoma (Table 2). The two latter tumors are listed equal to Lymphoma but are not primary tumors of orbit.

Out of lymphomas, 04 cases were non Hodgkin’s (two large and two small) and 02 were Hodgkin’s type. Our incidence was 13% but 7 – 20% has been reported in literature and same reported by Jawaid in his study⁵. However in Hodgkin’s type search for extra orbital involvement was not attempted. One case of orbital Burkitt’s tumor (B-cell) arising from ethmoid sinus was reported in a 5 year old boy⁶. In another report⁷ primary non Hodgkin’s Lymphoma which involved left orbit and upper lid in a 4 year old girl.

Editorial by Awan⁸ quoted 24 year study (1962 – 1986) about 750 orbital tumors although there was no mention of Burkitt’s tumor. He further stated that in early nineties 200 – 300 cases were reported annually and that such tumors were not recognized or misrecognized by the pathologists. Both Hodgkin’s and Non Hodgkin’s Lymphoma have been reported in immuno deficiency syndrome^9,10.

Out of 228 ophthalmic Lymphoma adenexal and ocular reported¹ during 1980 – 2005 more than 50% were located in orbit with rapid rising incidence. Complete remission or significant reduction of lymphoma lesions following antibiotic therapy for Chlamydia psittaca infection – suggest its role in aetiology of lymphoma¹¹. Some authors also noted higher incidence of non Hodgkin’s lymphoma in Asians than Europeans and blacks.

Relationship between lymphoma and Chlamydia psittaci with regard to aetiology and response to anti-bodies was previously reported by Ferri A et al in two separate papers with different team of coauthors^12,13.

Galieni et al.¹⁴reported fifteen patients with localized orbital lymphoma and low grade mucosa associated lymphoid tumor (MALT) which were treated with chemotherapy, radiotherapy and surgical excision with local relapse in three but disease spread was never recorded.

Hodgkin’s and Non Hodgkin’s classification depends on variable histological cellular pattern and morphology picture^13-15.

Non Hodgkin’s lymphomas are classified into B and T cells, B cells are much more common and consist of large B cells, small cells and marginal cells. Burkitt’s tumor is composed of large B Cells. Biopsy is sent to molecular diagnostic laboratory and following methods applied.

Immuno histochemistry with surface cell markers for B and T cells¹⁵.
Florometry

Genetic study (Chromosomal dependant) for definitive diagnosis and subtyping of lymphoma^16,17.

In our opinion, modern diagnostic techniques mentioned were non existent/non-available to pathologists of late 20^th century, could explain lack of proper histological diagnosis typing and subtyping.

CONCLUSION

The study revealed rising incidence and prevalence of orbital lymphoma. Its incidence was even higher than that of reported. Lymphoma tumor is localized, stationary, and occasionally assuming large size. Modern laboratory techniques have revealed higher incidence as reported in resent international study. Rising incidence and occurrence of this tumor need to be confirmed by further studies in future.

Author’s Affiliation

Prof. Faiz M. Halepota

Department of Ophthalmology

Muhammad Medical College,

Mirpurkhas, Sindh – Pakistan.

Prof. Khalid Iqbal Talpur

Chairman, Department of Ophthalmology

Liaquat University Eye Hospital Hyderabad,

Sindh – Pakistan

Dr. Mahesh Kumar Luhano

Clinical Pathologist, Department of ophthalmology

Liaquat University Eye Hospital Hyderabad,

Sindh – Pakistan

Late Sher Muhammad Shaikh

Department of Pathology

Chandka Medical College, Larkana

Sindh – Pakistan

Dr. Abdul Rehman Siyal

Department of Pathology

Muhammad Medical College, Mirpurkhas

Sindh – Pakistan.

ACKNOWLEDGEMENTS

Authors extend thanks to Dr. Aijaz Ali Khooharo, Associate Professor and Incharge Director, Advanced

Studies, Sindh Agriculture University Tandojam for his input in interpretation of results.

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