Clinicopathological Study of Orbito-ocular Lesions in Ninevah Province

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1 Clinicopathological Study of Orbito-ocular Lesions in Ninevah Province Shatha Th. Ahmad, Dept. of Pathology, Ninevah Medical College, Mosul University. Abstract Eye tumors that are malignant or cancerous originate in another area of the body and spread to the eye especially from the breast, lung, bowel or prostate. The current study was undertaken to determine the proportion of different types of oculo/orbital tumors among people living in Ninevah province in the north of Iraq. The present study has dealt with data from case files of (118) patients with or orbital and /or ocular tumor whom was treated surgically by enucleation or plastic surgery during the period from Jan 1993 up to Dec.2000 most of these tumors had underwent review of their histopathologial examination. Out of (118) cases (59) were malignant and(50) were benign,the rest were inflammatory. Out of (59 )cases malignant lesions, 18(30.5%) were BCC, with a mean age of 45.5 years,followed by SCC 17(28.8%) with a mean age of 59 years and then retinoblastoma 12(20.3%) most of them were under 6 years of age with a mean age of 2.5 years. Out of (50 )benign lesions (22) were in the conjunctiva and (25) in the eye lids,the rest were in the tissues surrounding the eye. Compound naevi were the most common lesion 18(36%) followed by dermoid &epidermoid 11 cases(22%),then squamous papilloma 8 cases(16%), then capillary hemangioma7(14%),the rest were different lesions. As in previously published studies, malignant lesions were more frequent in the eye lids (29)cases compared to conjunctival malignant lesions (9)cases only and the rest were intraocular. Whereas majority of conjunctival lesions were benign (22)cases. In conclusion, the high percentage of malignant lesions that were observed can likely to be attributed to elevated sun exposure and ultraviolet light in Iraq. Key words: Eye tumor, Orbito-ocular lesions Introduction Tumors of the eye can occur not only within the eye but in the eyelids and tissues surrounding the eye as well as its related structures and sometimes the patient with an orbital tumor may present first to the ENT clinic. Usually eye tumors that are malignant or cancerous originate in another area of the body and spread to the eye especially from the breast, lung, bowel or prostate. Two types of primary tumors arise within the eye itself and are retinoblastoma in children and melanoma in adults (1,2,3). Clinically, symptoms vary depending on the location of the ocular tumor. Often, the patient will first report changes in the visual acuity, diplopia, squint eyelid droop, fullness, discharge and/or pain. Retinoblastoma commonly present as leukocoria (White pupil) which is usually first noted by the family members and described as (cats eye appearance) (5,6). Early diagnosis and treatment are extremely important as eye tumors can spread via the optic nerve to the brain and via the blood stream to the rest of the body. Diagnosis can be achieved by physical examination by inspection of the external surface of the globes, eyelids and surrounding structures, then measurement of the visual acuity, visual fields, papillary responses, ocular motility. In some instances it is possible to remove the tumor surgically and still preserve vision,or by fine- tunes radiation therapy for precise focusing of radiation on the eye tumor (4,7). The current study was undertaken to determine the most common types of benign and malignant intraocular tumors in Ninevah province in Iraq, the mean age of distribution and their proportion in both sexes and the proportion in different parts of the eye and the eyelid and tissues surrounding the eye. Patient and Methods The present study has dealt with data from case files of (118) patients with or orbital and /or ocular tumor whom was

2 treated surgically by enucleation or plastic surgery at the AL-Zahrrawi teaching hospital and Al-Salam teaching hospital in Ninevah province who deal with eye problems beside AL-Zahrrawi private hospital during the period from Jan 1993 up to Dec.2000 most of these tumors had underwent review of their histopathologial examination. Age & gender specific rates were calculated. Results There were (118) patients enrolled for this study,59 (50 %)had malignant lesions and 50 (42.4%) had benign lesions, the remaining 9 (7.7%) had inflammatory lesions Table (1). Table (2) shows the different types of orbito-ocular tumors and the inflammatory lesions with their proportion. Table (3) shows the common types of malignant tumors. Out of the total 59 cases, 18(30.5%) were basal cell carcinoma (BCC), 17 (28.8%) were squamous cell carcinoma (SCC), 12 (20.3%) were retinoblastoma, 3 cases(5%)malignant melanoma, another 3 cases showed rhabdomyosarcoma, the rest showed 2 cases of NHL of small cell type one involving the eye ball &the other presented as supraorbital mass both as part of systemic lymphoma. Osteogenic sarcoma seen in 2 cases, and one case presented as recurrent conjunctival lesion showed carcinoma in situ. The last case was dermatofibro-sarcoma of the eye lid. Regarding the common types of benign lesions.out of total 50 cases,18(36%) were compound naevi. 8(16%)were squamous cell papilloma, 7(14%) dermoid cyst,another 7 cases(14%)were capillary hemangioma,the rest were epidermoid cysts, angiofibrolipoma, pilosebacious cysts,mucocele of maxillary sinus, meningothelial meningioma and the last one was a case of pleomorphic adenoma presented as a mass beneath the eye Table(4). The proportion of the malignant tumor according to the age and gender, (35) were females and ( 24 ) were males with a female to male ratio of 1.5: 1. All patients with retinoblastoma were children under 6 years of age,mean age was 30 months, youngest patient undergone enucleation was 7 months old Table(5). Mean age of patients with BCC was(54.9)years,females were as twice as males. Out of 18 cases of BCC.14(77.8%) were in the eye lid, 3 (16.6%) in the canthal region & one in the conjunctiva (5.6%). Regarding SCC mean age was 59 years female to male was 1.4:1. Out of the total 17 lesions, 11(64.7) were in the eye lid and 6 (35.3) in the conjunctiva. Table (6) shows comparative proportion rates of retinoblastoma in different localities in the world. Table (1): The frequency of different lesions of the eye. Lesion Female Male No. % No. % Malignant Benign Inflammatory Total Total No. % , %

3 Table (2): Common types of orbital and intraocular lesions Types number % Compound naevi Basal cell carcinoma Squamous cell carcinoma Retinoblastoma Squamous papilloma Capillary hemangioma Dermoid cyst Lacrimal gland inflammation 6 5 Epidermoid cyst Embryonal rhabdomyo- sarcoma Malignant melanoma NHL of small cell type Granuloma Angiofibrolipoma Osteogenic sarcoma Carcinoma in situ Pleomorphic adenoma Meningiothelial meningioma dermatofibrosarcoma Pilosabecious cyst Mucocele of maxillary sinus Persistant hyperplastic viteous Total

4 Table (3): Common types of malignant tumors. Type Number % Basal cell carcinoma Squamous cell carcinoma Retinoblastoma Malignant melanoma Embryonal rhabdomyo sarcoma NHL of small cell type Osteogenic sarcoma Carcinoma in situ Dermatofibrosarcoma Total Table (4): Common types of benign tumors. Type Number % Compound naevi Squamous papilloma 8 16 Dermoid cyst 7 14 Capillary hemangioma 7 14 Epidermoid cyst 4 8 angiofibrolipoma 2 4 Pilosabecious cyst 1 2 Mucocele of maxillary sinus 1 2 Meningiothelial meningioma 1 2 Pleomorphic adenoma 1 2 Total

5 Table (5): The proportion of malignant tumors according to the age & gender. Age BCC SCC Retino Blas-toma M.M Embryonal Rhabdomyo Sarcoma NHL others Total + > + > + > + > + > + > Total Table (6): Age-standard rates of retinoblastoma in children under age of 20 years (from international classification of childhood cancer ICCC, ) ISRAEL ISRAEL U.S (SEER) JORDAN EGYPT CYPRUS (ARAB) (JEWS) + > T + > T + > T + > T + > T + > T Discussion The eye & adjacent structures contain a wide variety of cell types and as one might expect, benign & malignant neoplasm arises from them. Intraocular neoplasms arises mostly from immature retinal neurons (retinoblastoma) and uveal melanocytes (melanoma) (1,2). The involvement of otolaryngologist beside the ophthalmologist may be needed sometimes as adjacent structures to the eye may be involved by the primary orbital lesion &the patient may first present to the ENT (3). Retinoblastoma is the most common ocular malignancy in childhood,the most common presenting sign is Leucocoria (white pupil) which is usually first noticed by the family members and described as a (cat's eye appearance) other cases present with strabismus or squint (1,2,3,4,5,6,7). However, several ocular conditions in childhood can clinically cause leucocoria in most cases by congenital cataract (35%). In any case of leucocoria,retinoblastoma has to be excluded without delay (6). In our study, out of 59 malignant eye tumors, 12 (20.3%) were retinoblastoma, the age ranged from 7 months-6years,the mean age was 30 months. While in U.S 300 cases of Rb pear year is diagnosed, in European children the highest proportion is seen in the first year of life (4,8,9). In Turkey, the clinical records showed that the mean age at the time of diagnosis of Rb was 25 months (10).. Rb in

6 Egypt, the rate of Rb per 1,000,000 and are age standardized to the world standard million and compared to other areas (tab 6) (11). In Yemen, Rb form 17% of malignant ocular tumors &this figure is similar to that recorded in our locality (13). In Nigeria the recorded proportion is higher (38.1%) and this may be attributed to the problems caused by inadequate facilities for diagnosis and management (14 ). When Rb occur in both eyes, it is always considered as hereditary ''familial'' type of the disease or sporadic in 60% of cases (1,2,3).In our series only one case out of 12 cases was bilateral, one eye was inoculated, the other was preserved to be treated by non-surgical methods. The major goal of treatment is to save the patient's life and secondarily to salvage the eye and vision if possible. Regarding malignant melanoma (MM), it is the most common primary intraocular malignancy. It may arise from melanocytes from any part of the eye.the choroids being the most common site (1,15,16) In Europe and US,the proportion of primary Intraocular melanoma is per million per year (16). Persons born in southern latitudes of the united states had a relative risk of 2.7 as compared with those born in the northern latitudes, this suggest that sunlight exposure in the south is an important risk factor. (16). Conjunctival melanoma is a condition of concern because of its rarity & lethal potential,it occurs only 1/40 as often as choroidal melanoma. It has no sex predeliction & found in middle aged & more in white people (17). In our series,the three cases were all choroidal melanoma &this goes with that recorded in other literatures (13,15). Regarding the conjunctiva,benign lesions were the most frequent 22 (37.3% ) and 8 (26,7% ) cases of malignant lesions. Out of( 22 )benign lesions, (17) cases were compound nevi (77.3) with mean age of (41.2) years, 2 cases of capillary hemangioma (9.1% ),another 2 cases of angiofibrolipoma (9.1% ) & one case of squamous papilloma (4.5%). Conjunctival nevus is a benign tumor most often located at the nasal,temporal limbus &rarely in the fornix (18). On the other hand, there was 6 cases(75%) of SCC,one case of BCC (12.5%)and one case of Ca in situ. In Tahran, Iran they recorded a higher proportion of malignant tumors especially SCC (25%) which can be attributed to elevated sun exposure & UV light in Iran (19). On the other hand,the eye lids were the most common localization for malignant tumors, 29 (53.7% ), benign tumors were the second most common 25 cases(46.3%). Most common malignant lesions were 17(58.6% )cases of BCC,11cases(38%)of SCC and one case of dermatofibrosarcoma (3.4%). In other literatures, BCC (rodent ulcer)was the most common malignant tumor of the eye lid (13,20,21,22). Benign lesions include 7cases (28%) of squamous cell papilloma, dermoid & epidermoid 11 cases (44% ), capillary hemangioma 5 (20%), one case of compound naevus (4%) and one case of pilosabecious cyst. Dermoid cyst usually present as external angular cyst although they may occur medially. Capillary hemangioma or ''strawberry nevus'',an abnormal growth of vascular tissue, is the most common vascular ocular tumor in children it appear at or soon after birth. Females outnumber male patients with hemangiomas by a ratio of 3:1 (23,24). While in our findings there were 4 males & 3 females. For squamous papilloma, form 16%of benign ocular lesions.in Yemen it formed 13% (13). In Tahran, Squamous cell papilloma form 7.8% (19). In Taiwan eye lid tumors, most common malignancy was BCC(65%) followed by SCC (12.6%), i.e. BCC dominates the proportion trends (22). Rhabdomyosarcoma arises most frequently in the head &neck &is predominantly a neoplasm of infancy &childhood &adolescence (5). Average age of patients affected by rhabdomyosarcoma is 7-8 years (25). In our series mean age for this tumor was 9 years. Regarding NHL, 2 cases where recorded one presented as supra orbital mass & the other as orbital tumor both were part of systemic NHL mean age 65 years one female & one male. In the reports from the national institute of health, the ocular(i.e. eye & adnexia) NHL rates /1oo,ooo person/year for both sexes were highest amongst

7 Asian/pacific Islands, lower in whites &lower still in blacks.proportion increased with advancing age &showed little difference in sex (26,27). NHL is one of the masquerade syndromes of malignant melanoma that can occur in 2 main patterns of presentations in the eye, metastatic involvement of uveal tract and primary involvement of the retina (27) As a conclusion, Eye cancer is a very deliberating disease,you can be born with it or acquire it later on. For families with history of eye cancer,preventive measures and early detection programs can help alleviate some anxieties and minimize damage. Some tumors are very aggressive and may present late with marked intraocular extension so that encleation of the eye is usually the treatment of choice because the major goal of treatment is to save the patient's life and secondarily to salvage the eye and vision if possible. References 1-Kumar V,Cotran RS,Robbins SL. Basic Pathology. W.B Saunders Company 6 th edit 1997;ch 7, Macsween PNM and Whaly K.Muir's Text book of pathology thirteen edit, New Delhi 1997, Bascom Palmer Eye Institute: Eye Tumors. html. 4-The Wilmer EyeInstitute at John's Hopkins. mer/about/ 5-Russel RCG,Norman S &Chri SJK. Baily & Love's Short Practice of Surgery 2 nd edit 2004,ch.45, Meier P,Sterker I,Tegetmeyer H. Leucocoria in childhood. Klin Monatsbl Augenheilkd,2006 :Jun;223(6): file://i:\entrez PubMed8.htm. 7-Ocular Tumors information complied by Manolette R.Roque, last updated on Nov file://i:\eye tumors 2.htm 8-From children care of Atlanta At fault.aspx?id= MacCarthy A,Draper GJ,Steliarova FE,Kingston JE.Retinoblastoma proportion and survival in Europian children( ).report from the Automated Childhood cancer information system Project. Eur J cancer,2006 Sep;42(13): Ozkan A,Pazarli H,Celkan T et.al. Retinoblastoma in Turkey :survival and clinical characteristics Pediatr Int,2006 Aug;48(4): file://i:\ Entrez PubMed4.htm 11-Ayhan O Cavdar,Tezer K. International Classification of childhood cancer (ICCC) from Retinoblastoma by Roperter :Danielle Banks, At Quick links file://i:whsv- News.htm. 13-Al-Samarrai Abdul Razzak.Al Wazir I.Clinico-pathological Study of Orbito-ocular tumors in Yemen.Iraqi Med J,2000:vol.49 ; Onwasigwe EN.Orbito-Ocular Tumors In Nigerian Children. Int College of Medicine,2002:vol 7(2); Eye or Orbital-Ocular Melanoma. ppi/ Types of Cancer/ Eye or Orbital/ Ocular Melanoma/ default.htm. 16-Tucker MA,Shields JA,Hartge P et. Al. Sun exposure as risk factor for intraocular malignant melanoma.the New England Journal Of Medicine.1985:vol.313: no.13; Bbrownstein S. Malignant Melanoma of the Conjunctiva.From Cancer Control :Journal of the Moffitt Cancer Center.file://I:\Malignant Melanoma of the Conjunctiva.htm. 18-Casol LSH,Airey F,Arman M,Jerry A. Conjunctival naevi :clinical features and Natural course in 410 consequative patients.arch Opthalmol,Feb 204;122: Amoli FA,Heidari AB.Survey of 447 patients with conjunctival neoplastic lesions in Farabi Eye Hospital, Tehran, Iran. Opthalmic Epidemiology,2006 Aug ;13(4): Klin Oczna.Tumot and Tumor-like lesions of eyelids collected at Department of Pathological Anatomy,Wroclaw Medical University, between 1946 and ;107 (7-9): 475-8

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