Orbital Tumors - A Clinico Pathological Study

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1 Orbital Tumors - A Clinico Pathological Study Radha. J. DO, Ani Sreedhar. MS. Little Flower Hospital, Angamaly, Kerala ORIGINAL ARTICLES Abstract: Aim. To study the clinical and histopathological profiles of orbital tumors and its surgical management and frequency of biopsy proven orbital tumors. Materials and Methods Retrospective study of 24 patients with orbital tumors who presented to Little Flower Hospital, Angamaly during a period of Jan May 2005 subjected to orbitotomy was done..samples for histopathological diagnosis were obtained either through incisional or excisional biopsy. Results Lymphoma was the most common malignant Tumor.The results correlated well with the previously conducted studies. Conclusion Relative incidence of malignant tumors is seen commonly in elderly while no substantial change was noticed in the incidence of other tumors. Orbital tumors represent varied group of lesions, including primary, secondary and metastatic tumors The reported incidence of orbital mass lesions varies widely from series to series, depending on the source of material studied Orbit is an anatomically complex structure containing globe, extra ocular muscles, and fat, vascular, nervous and glandular connective tissues. All these anatomical structures can give rise to neoplasms. The aim of this study is to analyze the demography, clinicopathological profiles, surgical management and frequency of biopsy proven orbital tumors Although this is a small study group, it does represent patients who came to our department with space occupying lesions suspicious enough to prompt a biopsy. Materials and Methods Retrospective analysis of patients who presented to Little Flower Hospital with a clinical diagnosis of orbital tumor between January 2003 and May 2005 was done A total of 24 patients were studied. Patient data (age. gender, chief complaints and duration of symptoms). Ocular examination findings (laterality.. BCVA, EOM restriction, proptosis and its type. displacement of globe, palpable mass, location of mass) were noted. All these patients underwent CT scan of the orbit as a part of investigation. Pathological diagnosis was obtained by either incisional or excision biopsy through various approaches. Post operative status namely visual acuity, pupils and EOM were also noted. Results: Total of 24 patients were studied. Out of them 15 (62.5%) were male and 9 (37.5%) were female [Table-1]. Age at presentation ranged from 4.5 months 1o 76 yrs, median age group being 49.5 yrs [Table-2] Table 1 Sex distribution Sex Number Percentage Male Female Table Age distribution Age distribution September

2 Age group % of cases Table 3 Clinical features Symptoms /Signs Number Percentage (n 24) Proptosis Palpable mass Defective vision Restricted Motility 6 25 Ptosis Most common presentation was unilateral proptosis (833%). There was a palpable mass in 62.5% of cases, and restriction of motility in 25% of cases. Two patients presented with ptosis (8.3%). Table 4 Histological Classification Overall, lymphoid tumors were the most common Tumors in our study (33.33%) followed by hemangiomas (16.66%) and lacrimal gland tumors (8.4%). Malignant tumors accounted for 29.2% of cases among which lymphoma was the most common (20,83%) and benign lesions for 70.8% among which hemangiomas were most common (23.5%). Lymphoid tumors accounted for 8 out of 24 cases (33.33%) and the most frequent tumor in this group were malignant lymphomas (5 cases; 20.83%), out of which 80% were MALTomas (mucosa associated lymphoid tissue) and only one case had involvement of spleen indicating secondary involvement of orbit. Cases of reactive lymphoid hyperplasia (3 Cases, 12.5%) also were diagnosed based entirely on histopathological diagnosis. 75% of these tumors occurred m males and 25 % in females. The median age of involvement in RLH was 47 yrs while that in malignant lymphoma was 65 yrs. Quadrant of involvement was superonasal in 3 patients, superotemporal in 3 patients, superior quadrant in 1 and inferior quadrant in 1 patient. Category No of % of total Age range patients Lvmphoid tumors * Reactive Iymphoid hvperplasia (RLH) * Maligrant Lymphoma Vasculogenic /12-64 * Hemangioma /12-52 * Lymphangioma * Hemangiopericytoma Lacrimal gland tumors I&52 * Pleomorpthic adenoma * Adenoid cystic carcinoma Cystic tumors * Epidermoid &40 * Dermoid 1 4,2 36 * Mucocele Inflammatory 3 1LS * Idiopathic 1 4,2 39 * Parasitic granuloma &75 Secondary from SCC conjunctiva Quadrant involved No of cases Superonasal 3 Superotemporal 3 Superior 1 Inferior 1 Hemangiomas were the next most common of the orbital tumors (16.6%) and the most common benign variety (25%), Out of the 4, 3 were cavernous variety with mean age of presentation being 41.3yrs. The one with capillary variety presented in 41/2 months old child. The sex distribution was equal (50% in each group). In all cases of cavernous variety, CT scan revealed a well defined enhancing mass mostly occupying retro bulbar space. The capillary variety occurred in the floor of the orbit. Lymphangioma was seen in a 3 yr old child who presented with bluish mass in inferior quadrant. During surgery it was found to be filled with chocolate colored fluid. Hemangiopericytoma was seen in 1 patient involving September

3 superomedial quadrant. During surgery it was so friable that it could be excised only in part- 3 patients had lacrimal gland tumors. Out of them 2 had epithelial variety - one benign (Pleomorphic adenoma) and one malignant (adenoid cystic carcinoma). One patient had non-epithelial tumor (MALTomas) and so was included in the lymphoma group- All these patients presented with non axial proptosis. CT scan showed bony erosion with intra cranial extension in the patient with Adenoid cystic carcinoma. Among the benign cystic tumors, 2 were epidermoid cysts, one Dermoid and one mucocele. Epidennoid cyst showed equal sex distribution (50 %) and both the cases involved the supero nasal quadrant. CT scan showed a well defined mass in the mid orbit in both these cases. 2 patients had parasitic granuloma. Both of them presented with periorbital edema and ptosis, HPE of these excised tissue revealed Dirofilaria, a nematode. Idiopathic inflammatory pseudo tumor was observed in 1 patient. The patient presented with a subconjunctival mass involving medial rectus and associated CRVO. CT scan showed MR mass with bulky muscle and lacrimal gland. One patient had orbital extension of a SCC conjunctiva. Mass was excised and post op radiotherapy given. Lesions were approached either through an anterior orbitotomy (21 cases; 87.5%) or lateral orbitotomy (3 cases; 12 5%) Table 6 Orbitotomy approaches APPROACHES NO OF PATIENTS % ANTERIOR Conjunctival Skin 6 25 Lid splitting Swinging lid LATERAL DISCUSSION: Orbital tumors can occur at various age groups, and because of their myriad presentation, their diagnosis pose a great challenge. Although imaging studies graphically illustrate tissue definition, pathological conditions can be assessed definitely only by obtaining tissue specimen surgically. This was accomplished by excision biopsy (well defined lesions) and incision biopsy (ill defined lesions), In our study of 24 patients, lymphoid tumors were found to be most common accounting for 33.3% of cases. Lymphomas of ocular adnexa (conjunctiva, orbit and lacrimal gland) account for 8 % of all extra nodal lyrnphomas They form one end of the spectrum of lymphoprojiferative lesions that are seen at these sites; other end being reactive lymphoid hyperplasia, formerly termed benign lymphoma or pseudo lymphoma 2. Orbital lymphomas have worse prognosis than conjunctival lymphomas 3 4. In our study 62.5% of patients had malignant lymphoma while 37 5% had RHL. Males were more frequently affected in our study (75%) in contradiction to other studies where females were more commonly involved 6. 80% of lymphomas in our study were primary MALT tumors. Only one patient had involvement of spleen. Demirci et al 17 have showed that this neoplasm has a predilection for older patients and it is the most common neoplasm in older adult group. Among the benign lesions, hemangioma was found to be the most common, accounting for 16.66% (4cases). Cavernous hemangiomas are the most frequent among vascular tumors accounting for 50 % of all vascular tumors and 13.5 % of all orbital lesions. Shields et al 7 found in their studies that these are the most common benign orbital tumors in adults, 7, 8 with usual age of presentation at yrs. Typically they are located in the retro bulbar space. They are most commonly approached through the lateral orbitolomy because of their usual location in the muscle cone. Capillary hemangiomas accounted for 16-66% of all vascular tumors and 12.5 % of all orbital lesions. They occur almost exclusively in children 9. In our series there was one case in a 4 ½ months old child. They usually progress during the first few months of life, but then stabilizes and later on regress by puberty. Anteriorly located circumscribed capillary hemangjomas can be managed by complete excision. Studies have shown that when confined to deep orbital tissues^ they may cause asymmetric refractive errors, which tend to persist even after regression of hemangioma unless early treatment is instituted. High incidence of amblyopia also occurs 11, 12. Lymphangiomas accounted for 16.66% of all vascular tumors and 12.5 % of all orbital lesions. They most often present during childhood. In our study a 3 yrs old child presented with proptosis. HPE. revealed chocolate cyst- These cysts are circumscribed brown lesions and are September

4 sequelae of hemorrhage. This cyst can cause optic nerve compression and visual impairment necessitating emergency surgery- Surgical removal or debulking is difficult. Hemangiopericytoma generally affect adults- They are exceedingly friable and at surgery they may bleed extensively. Hence complete removal is technically difficult. Lacrimal gland tumors accounted for 8.4% of all orbital lesions. Of the 3 cases of lacrimal gland tumors, % were of epithelial origin (50% benign and 50% malignant) and 33.3% of non epithelial origin that was a primary MALT lymphoma included in the lymphoma group. Lacrimal gland tumors are classified as Epithelial 1) Pleomorphic adenoma 2) Pleomorphic adenocarcinoma 3) Adenoid cystic carcinoma. Non epithelial 1) Pseudo tumor 2) NHL (Non Hodgkin s Lymphoma) 3) RLH Benign epithelial tumors are generally common 1U. We had two cases of epithelial tumors (1 benign and 1 malignant) and one case of non epithelial tumor (MALT). Although pleomorphic adenomas are histologically benign, incomplete excision will lead to relentless recurrences and even malignant transformation 13, 14. Hence when suspected, lateral orbitotomy is mandatory and entire tumor must be excised enbloc 13, 15. Majority of malignant tumors are adenoid cyst carcinoma- Major cause of death is intracranial spread hastened by perineural invasion. Treatment is exenteration and post op radiotherapy. Inflammatory lesions accounted for 12.5% of all orbital lesions among which pseudo tumor accounted for 4.2% and parasitic granuloma for 8.3%. Accurate diagnosis was established only by histopathology. Orbital pseudo tumor is one distinct disease albeit with many clinical and histopaihological guises r6. Hence it is a diagnosis of exclusion. It can occur in any age and sex 3 Parasitic granulomas are generally rare. But we had two cases presenting with periorbital edema. Histopathology revealed Dirofilaria, a nematode. Cystic lesions accounted for 16.66% of all orbital tumors. Dermoid accounted for 25% of all cystic lesions and 4.2% of all orbital tumors, Epidermoid accounted for 50% of all cystic lesions and 8.3% of all orbital tumors. Dermoid and Epidenmoid cysts are the most common cystic tumors of the orbit Mucocele accounted for 25% of all cystic lesions and 4 2% of all orbital tumors. It most commonly arises from the frontal or ethmoidal sinus and may erode the orbital roof producing proptosis. Secondary involvement of orbit from adjacent structures arises most commonly from orbital extension of uveal melanoma followed by conjunctival melanoma 10. We had one case of secondary involvement from SCC of conjunctiva. To sum up, this study was undertaken to determine the number and percentage of various lesions presented to our Table7 Published series of orbital tumors in adult population Classification Shields B Henderson 18 Rootman 19 Wilson 20 Kennedy 21 Seregard 22 Lyrnphoid Vascular Lacrimal gland B Cystic Inflammatory Secondaries <1 1 <1 1 <1 1 Total September

5 hospital. Based on the results of our study, most common orbital tumors include lymphoid tumors ( %), cavernous hemangiomas (125%), inflammatory lesions (12.5%) and cystic lesions (12.5%). The relative incidence of malignant tumors is more in the elderly. No substantial change occurred in the relative incidence of inflammatory, vascular or cystic tumors. These figures correlated with the previous studies conducted by Demirci et al 17 The following table shows the frequency of orbital tumors in general population established through various studies. CONCLUSION: With knowledge of incidence of various tumors and pseudotumors in this series, combined with improvements in imaging techniques the clinician should be able to arrive at a fairly accurate diagnosis in most cases, which in turn facilitates decision-making regarding management- The patient s age should be kept in mind while evaluating a patient with possible orbital malignancy. Availability of CT arid MRI however should not neglect necessity of careful history, systemic and careful examination. r September

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