Annals of African Medicine Vol. 5, No. 3; 2006: 129 – 131

Orbito-Ocular Malignant Tumours in Zaria, : A 10-Year Review

1A. Mohammed, 1S. A. Ahmed, 2N. E. Ahmedu and J. S. Maisamari

1Department of Pathology, University Teaching Hospital, Zaria and 2National Eye Centre, , Nigeria Reprint requests to: Dr. A. Mohammed, Department of Pathology, Teaching Hospital, Zaria, Nigeria. E-mail: [email protected]

Abstract Background: Malignant orbito-ocular tumours are common, frequently affect the young and cause morbidity and mortality. There was no prior published report from our centre. Method: This study was a ten-year retrospective histopathological analysis of orbito-ocular malignant tumours seen at the Department of Pathology of the Ahmadu Bello University Teaching Hospital Zaria (January 1993 to December 2002). Histopathology diagnosis of specimens of the eye and adnexae were retrieved from the laboratory bench books and relevant information on the age of the patient and histopathologic type were analysed. Results: A total of 124 orbito-ocular malignant tumours were recorded. There were 2480 malignant tumours and these constitute 5% of all malignant lesions seen. The most frequent histological types encountered were retinoblastoma (40.3%), squamous cell carcinoma (33.1%) and Burkitt’s lymphoma (9.6%). There was equal sex distribution and infants were affected in 49.2% of cases with a mean age of 4.5 years. The most common sites in 82 cases were intraocular (54.8%), the orbit (23.2%) and conjunctiva in 17.1% of cases. Conclusion: Malignant orbito-ocular tumours are not uncommon in this environment and frequently affect infants; therefore appropriate medical facilities should be put in place to aid in diagnosis and management of these lesions.

Key words: Orbito-ocular, malignancies

Résume Arrière-plan : Les tumeurs malignes orbito-ocullaires sont fréquent et affectent les jeunes et est une cause de mortalité et de morbidité. Aucune étude n’a été publiée de ce centre. Méthode : Cette étude est une analyse histopathologique rétrospective, des tumeurs malignes orbito- ocullaires vues au Département d’Anatomie Pathologie du centre Hospitalier Universitaire Ahmadu Bello Teaching Hospital Zaria, durant dix ans, de Janvier 1993 à Décembre 2002. Les données utilisées ont étaient récupérées de la banque des données du Département d’Anatomie Pathologie et les informations telles que l’age des patients et le type de cancer a été analysé. Résultats : Un total de 124 tumeurs orbito-ocullaires a été enregistré sur un total de 2480 tumeurs vues durant la période d’étude. Ceci représente 5% de toutes les tumeurs malignes. Le type histologique fréquemment rencontre fut le retinoblastoma (40.3%), les carcinome cellulaire squameux (33.1%), et les lymphome de Burkitt (9.6%). La distribution entre sexe été égale et les enfants étaient les plus affectes, 49.2% de toute les tumeurs avec une age moyen de 4.5 ans. Les sites les plus affectés fut la conjonctive 17.1% des cas, l’orbit 23.2% des cas et œil intraoculaire 54.8%. Conclusion : les tumeurs orbito0ocullairs ne sont pas rare dans cette région et affectent fréquemment les enfants, il est donc nécessaire de mettre sur place des structures sanitaire capable de diagnostiquer et de prendre en charge ces lésions.

Mots clés : orbito-ocullaires, malignes

Introduction mortality.1 - 3 Visual loss in children has implications for all aspects of the child’s development.4 Orbito-ocular malignancies are unsightly and may The most common histopathological types are contribute to visual disturbances. Although thought to retinoblastoma, squamous cell carcinoma and be rare, they contribute to significant morbidity and Burkitt’s lymphoma.5, 6

130 Orbito-ocular malignant tumours. Mohammed A. et al.

Materials and Method Table 3: Anatomical site of 82 orbito-ocular malignancies This study was a ten-year retrospective histopathological analysis of orbito-ocular malignant Anatomical site No. (%) tumours seen at the Department of Pathology, Intraocular 45(54.8) Ahmadu Bello University Teaching Hospital Zaria, Orbit 19(23.2) Nigeria (January 1993 to December 2002), using the Conjunctiva 14(17.1) laboratory bench book. Eyelid 3(3.6) Lacrimal gland 1(1.2) Total 82(100) Results

One hundred and twenty four (5%) orbito-ocular Discussion malignancies were seen out of a total of 2480 malignancies. One hundred and twenty four malignant orbito-ocular Retinoblastoma which accounted for 50 (40.3%), tumours were analysed during a ten-year period. This squamous cell carcinoma 41 (33.1%) and Burkitt’s constituted 5% of all malignant tumours seen in the lymphoma 12 (9.6%) of the cases (Table 1). department during the study period. This figure The M:F ratio was 1:1, with a peak age in the first however is higher than the 2.2% reported in Ibadan5 decade (mean 4.5 years) (Table 2). The pattern of and 3.3% in Kampala.7 anatomic distribution of 82 cases is shown in Table 3. Retinoblastoma was the commonest tumour The commonest site was intraocular 45 (54.8%), encountered which constituted 40.3%, and this is orbital in 19 (23.2%) and conjuctiva in 14 (17.1%). followed by squamous cell carcinoma (33.1%) and Burkitt’s lymphoma in 9.6% of cases. The predominance of these three malignancies are similar Table 1: Histology of orbito-ocular malignancies in with reports from Kaduna and Uganda5, 8and several 124 patients reports from .7, 9, 10 This contrasts a report from Ile- where no case of orbito-ocular Burkitt’s Histological diagnosis Total (%) lymphoma was recorded in a ten-year study.1 Basal Retinoblastoma 50(40.3) cell carcinoma and choroidal melanoma which Squamous cell carcinoma 41(33.1) constituted 2% each in this report contrasts a 6 Burkitt’s lymphoma 12(9.6) Caucasian report which showed it to be common and 9, 10 Rhabdomyosarcoma 4(3.2) is similar to other reports in Nigeria. Lymphoma(other) 3(2.4) Retinoblastoma occurs in sporadic and familial Adenocarcinoma 4(3.2) forms, both require mutation involving the Rb gene located on chromosome 13q14.11 However, Burkett’s Astrocytoma 2(2.6) lymphoma has been well characterized in malarial Melanoma 2(2.6) endemic regions and it involves translocation at Basal cell carcinoma 2(2.6) 14q32 following complex mechanisms in B – cell Kaposi sarcoma 1(0.8) lymphocytes.12 Lacrimal gland carcinoma 1(0.8) The equal sex distribution observed in this study is Metastatic nasopharyngeal carcinoma 1(0.8) comparable to studies from Ile-Ife1 and Ibadan, 5, 10 but Osteogenic sarcoma 1(0.8) contrasts with the male to female ratio of 3.4:1 from 13 Total 124(100) , North Eastern Nigeria. Orbito-ocular malignancies are found to be commoner within the first decade with a mean age of 4.5 years. This conforms to previous reports from Ile-Ife, Maiduguri Table 2: Age and sex of 124 patients with orbito- 1, 5, 13 ocular malignancies and Ibadan. The predominant site of orbito-ocular malignancies in 82 cases was intraocular constituting Age M F Total (%) 54.8%, followed by the orbit in 23.2% and 0 - 10 31 30 61(49.2) conjunctiva in 17.1% of cases. Malignancies of the 11 -20 7 5 12(9.7) lacrimal glands are rare accounting for 1% of cases. 21 - 30 9 10 19(15.3) No case of eyelid malignancy was seen in this study. 31 - 40 9 5 14(11.3) This report however agrees with a study in Ibadan by 4 41 - 50 3 7 10(8.1) Olurin et al. 51 - 60 0 3 3(2.4) One case of uveal melanoma (2.6%) was encountered in this study. Another African5 study 61 - 70 2 2 4(3.2) show similar trend; where 3.7% of Ocular melanoma 71 – 80 1 0 1(0.8) was reported. While reports from Caucasian series Total 62 62 124(100) show that melanoma arising from the pigmented

Orbito-ocular malignant tumours. Mohammed A. et al. 131 or potentially pigment producing cells of the uvea are in Africans of Uganda. Cancer 1967; 20: 1689 – the most frequent primary intraocular neoplasms in 1698 adults; and can occur in children and adolescents, 8. Abiose A, Adido J, Agarwal SC. Childhood even in neonates.14, 15 Most of them arise from pre- malignancies of the eye and orbit in Northern existing benign nevi.16-18 Nigeria. Cancer 1985; 55: 2889 – 2893 Orbito-ocular malignancies are common and 9. Ajaiyeoba IA, Pindiga UH, Akang EE. Tumours frequently affect infants. Retinoblastoma, squamous of the eye and orbit in Ibadan. East Afr Med J cell carcinoma and Burkitt’s lymphoma are the 1992; 69: 487 – 489 commonest histopathological types encountered and 10. Bekibele CO, Oluwasola AO. A most of these malignancies are intraocular. Further clinicopathological study of orbito-ocular researches into the clinical behaviour and diseases in Ibadan between 1991 – 1999. Afr J management options of these malignancies in Med Sci 2003; 32: 197 – 202 Nigerian patients should therefore be encouraged. 11. Friend SH. A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma. Nature 1986; Acknowledgement 323: 643 12. Diseases of childhood and infancy. In: Cotran We are grateful to Mrs. Aishetu Umar of Mathematics and Mr. Raoul Dasi of Pathology Departments, for preparation of the RS, Kumar V, Robbins SL (eds). Robbin’s manuscript. pathologic basis of disease. Saunders, Philadelphia, 1994; 241 – 303 13. Otoh EC, Johnson NW, Danfillo IS, et al. References Primary head and neck cancers in North Eastern Nigeria. West Afr J Med 2004; 23: 305 – 313 1. Amusa YB, Olabanji JK, Ogundipe OV, et al. 14. Barr CC, Mclean JV. Zimmerman LE; Uveal Pattern of head and neck malignant tumours in a melanoma in children and adolescents Arch Nigerian teaching hospital – a ten year review. opthalmol 99; 2136, 1981. West Afr J Med 2004; 23: 280 – 285 15. Broadway D, Lang S, Harper J, et al. Congenital 2. Neal AJ, Hoskin PJ. Head and neck malignant melanoma of the eye. Cancer 1991; 67: malignancies. In: Clinical oncology. 1997; 133 – 2642-2652 141 16. Yanoff M, Zimmerman LE. Histogenesis of the 3. Hearly GB. Malignant tumours of the head and malignant melanomas of the uvea I. Nevi of neck in children: diagnosis and treatment. choroids and ciliary body. Arch opthamol 1966; Otolaryngol Clin North Am 1980; 13: 483 – 488 76: 784-796 4. Gilbert CE, Forster A. Childhood blindness in the 17. Yanoff M, Zimmerman LE. Histogenesis of the context of VISION 2010 – the right to sight. Bull malignant melanomas of the uvea II. The WHO 2001; 79: 227 – 232 relationship of the uveal nevi to malignant 5. Olurin O, Williams AO. Orbito-ocular tumours in melanomas. Cancer 1967; 20:493-507 Nigeria. Cancer 1972; 30: 580 – 586 18. Yanoff M, Zimmerman LE. Histogenesis of the 6. Clemmenson J. Statistical studies in malignant malignant melanomas of the uvea III. The neoplasms. Munksgaard, Copenhagen, 1965; 34 – relationship of congenital ocular malanocytosis 40 and neurofibromatosis to uveal melanomas. Arch 7. Templeton AC. Tumours of the eye and adnexa opthamol 1967; 77: 331-336