Background: Retinoblastoma is the commonest intraocular malignancy in childhood. The national
epidemiological characteristics of retinoblastoma in Mozambique are not clearly known. Early diagnosis
and appropriate treatment leads to a favourable outcome while a delayed diagnosis and inappropriate
treatment can be fatal.
Objective: To determine the pattern of presentation and management of retinoblastoma patients at
Maputo Central Hospital during a 5 year period (1st January 2010 to 31st December 2014).
Methodology: The study was a retrospective case series carried out at Maputo Central Hospital
(Ophthalmology and Paediatric Oncology Services). Records of all retinoblastoma patients were retrieved
with the help of the specific file number. Demographic characteristics, clinical presentation, investigations
and management modalities of retinoblastoma patients were retrieved. Data was extracted and entered
into a structured questionnaire and analysed.
Results: A total of 57 patients from 7 different provinces of Mozambique had clinical, histological or both
clinical and histological diagnosis of retinoblastoma. The overall mean age at presentation was 30.5 months
(CI 24.65 - 36.05) (SD 21.5); 82.4% of cases had unilateral retinoblastoma while 12.3% of cases had bilateral
(mean age at presentation was 30.2 months and 26.4 months respectively). The female: male ratio was
1.2:1. The main presenting complaint was white reflex (62.5%) followed by eye swelling (50%), eye redness
(40%) and poor vision (32.5%) while the main clinical signs were leukocoria (55%) followed by proptosis
(50%) and eye redness (27.5%). Out of the 51 eyes who had surgery: 96% did not have documentation
of the choroid and 98% of the scleral, but 54.9% of the eyes had optic nerve involovement, 33.3% had
resection margin involvement and 31.4% had periocular tissue involvement. The main modalities of
management were enucleation (93.9%), chemotherapy (24.5%) and exenteration (18%). The eligible
patients for chemotherapy were 67.7% but only 35.3% of the patients received chemotherapy.
Conclusion: Many patients presented at older ages (with a mean age at presentation of 30.5 months) and
many presented late with advanced orbital disease. Majority of the patients had unilateral retinoblastoma.
Significant number of patients had optic nerve, resection margin and periocular tissue involvement noted
on histology. The main modalities of management were enucleation, exenteration and chemotherapy,
although there were no clear eligibility criteria for chemotherapy in retinoblastoma patients.
Ophthalmologists, American Academy of
Ophthalmic Pathology and Intraocular Tumours:
Section 4 – BCSC: AAO, 2012 – 2013
MacCarthy A, Birch JM, Draper GL, et al.
Retinoblastoma: treatment and survival in Great
Britain 1963 to 2002. Br J Ophthalmol. 2009;
Nyawira G, Kahaki K, Kariuki M. Survival
among retinoblastoma patients at KNH, Kenya.
J Ophthalmol Eastern Central and Southern Afr.
; 17(1): 15-19.
Honavar SG. Retinoblastoma: They Live and
See! AIOS ... MD, FACS Head, Ocular Oncology
Service, LV Prasad Eye InstituteLV Prasad Marg,
Banjara Hills, Hyderabad 500034, India.
Shields CA, Shields JA. Recent development
in the management of retinoblastoma. J Pediat
Ophthalmol Starbismus. 1999; 36: 8-18.
Shields JA, Parsons H, Shields CL, et al. The
role of cryotherapy in the management of
retinoblastoma. Am J Ophthalmol. 1989; 108:
Shields CL, Mashayekhi A, Sun H. Iodine 125
plaque radiotherapy as salvage treatment for
retinoblastoma recurrence after chemoreduction
in 84 tumors. Ophthalmology. 2006; 113(11):
Demirci H, Shields CL, Meadows AT, et al. Longterm
visual outcome following chemoreduction
for retinoblastoma. Arch Ophthalmol. 2005; 123
Antoneli CB, Ribeiro KC, Steinhorst F, et al.
Treatment of retinoblastoma patients with
chemoreduction plus local therapy: Experience
of the AC Camargo Hospital, Brazil. J Pediat
Hematol Oncol. 2006; 28 (6): 342-345.
Chan HS, Gallie BL, Munier FL, Popovic BM.
Chemotherapy for retinoblastoma. Ophthalmol
Clin North Am. 2005; 18 (1):55-63.
Shields CL, Shields JA, Needle M, et al. Combined
chemoreduction and adjuvant treatment for
intraocular retinoblastoma. Ophthalmology. 1997;
Friedman DL, Himelstein B, Shields CL, et al.
Chemoreduction and local ophthalmic therapy for
intraocular retinoblastoma. J Clin Oncol. 2000;
Wilson MW, Rodriguez-Galindo C, Haik
BG, et al. Multiagent chemotherapy as
neoadjuvant treatment for multifocal intraocular
retinoblastoma. Ophthalmology. 2001; 108 (11):
ElZomor H, Taha H, Aleieldin A, et al. High
risk retinoblastoma: Prevalence and success of
treatment in developing countries. Ophthalmic
Genetics. 2015; 36(3): 287-289.
Uusitalo MS, Van Quill KR, Scott IU, et al.
Evaluation of chemoprophylaxis in patients with
unilateral retinoblastoma with high-risk features
on histopathologic examination. Arch Opthtalmol.
; 119 (1) 41-48.
Nyamori JM, Kimani K, Njuguna MW, Dimaras H.
The incidence and distribution of retinoblastoma
in Kenya. Br J Ophthalmol. 2012; 96: 141-143.
Bowman RJC, Mafwiri M, Luthert P, et al.
Outcome of retinoblastoma in East Africa Pediat
Blood Cancer. 2008; 50:160–162.
Makite I, Kimani K, Njuguna MW. Delay in
presentation and management of retinoblastoma
patients at Kenyatta National Hospital.
Dimaras H, Kimani K, Dimba EAO, et al.
Retinoblastoma. The Lancet. 2012; 379 (9824):
Dimaras H, Dimba E, Gallie BL. Challenging the
global retinoblastoma survival disparity through
a collaborative research effort. Br J Ophthalmol.
; 94: 1415–1416.
Chang CY, Chiou TJ, Hwang B, et al.
Retinoblastoma in Taiwan: survival rate and
prognostic factors. Japan J Ophthalmol. 2006;
Nyaka AS, Kimani K, Kollmann MK. The pattern
of retinoblastoma at Queen Elizabeth Central
Hospital, Malawi. University of Nairobi. M. Med
Ophthalmology Thesis, 2010.
Waddell KM, Kagame K, Ndamira A, et al.
Clinical features and survival among children
with retinoblastoma in Uganda. Br J Ophthalmol.
Broaddus E, Topham A, Singh AD. Incidence
of retinoblastoma in the USA: 1975-2004. Br J
Ophthalmol. 2009; 93:21-23.
Owoeye JFA, Afolayan EAO, Ademola-Popoola
DS. Retinoblastoma – a clinicopathologic study
in Ilorin Nigeria. Afr J Health Sci. 2005; 12(3-4):
Kimani K, Ilako D, Kollmann M. A review of
retinoblastoma, presentation, diagnosis and
management at Kenyatta National Hospital.
University of Nairobi, 2000 (Unpublished).
Roth DB, Scott IU, Murray TG, et al. Echography
of retinoblastoma: histopathologic correlation
and serial evaluation after globe-conserving
radiotherapy or chemotherapy. J Pediat
Ophthalmol Strabismus. 2001; 38:136–143.
Graaf P, Göricke S, Rodjan F, et al. Guidelines
for imaging retinoblastoma: imaging principles
and MRI standardization. Pediat Radiol. 2012;
Khelfaoui F, Validire P, Auperin A, et al.
Histopathologic risk factors in retinoblastoma:
A retrospective study of 172 patients treated in a
single institution. Cancer. 1996; 77:1206–13.
Magramm I, Abramson DH, Ellsworth RM.
Optic nerve involvement in retinoblastoma.
Ophthalmology. 1989; 96: 217–222.
Chan HS, DeBoer G, Thiessen JJ, et al.
Combining cyclosporin with chemotherapy
controls intraocular retinoblastoma without
requiring radiation. Clin Cancer Res. 1996; 2 (9):