Retinoblastoma in a pediatric oncology reference center in Southern Brazil

Abstract

Background: Retinoblastoma (Rb) is the most common intraocular tumor diagnosed in children in Brazil. However, detailed information is lacking regarding patient clinical demographics. This study aimed to determine the clinical profile of patients with Rb who were treated in a public university hospital in southern Brazil from 1983 to 2012. Methods: Patients' medical records were reviewed to retrospectively identify patients with a principal diagnosis of Rb. Rb was classified as hereditary or non-hereditary. Clinical staging was reviewed by an ophthalmologist. Statistical analysis was performed using SPSS. Results: Of 165 patients with a diagnosis of Rb during this period, 140 were included in the study. Disease was unilateral in 65.0 % of patients, bilateral in 32.9 %, and trilateral in 2.1 %. The mean age at onset of the first sign/ symptom was 18.1 month, and 35.7 % of patients were diagnosed during the first year of life. The most common presenting signs were leukocoria (73.6 %) and strabismus (20.7 %). The mean age at diagnosis was 23.5 months, and time to diagnosis was 5.4 months. In patients with clinical features of hereditary Rb, both onset of the first sign/symptom and diagnosis were at an earlier age than in patients without these features (12.3 vs 21.6 months [P = 0.001] and 15.9 vs 28.0 months [P < 0.001], respectively). However, there was no significant difference in overall survival between the two groups. Ocular stage at diagnosis was advanced in 76.5 % (Reese V) and 78.1 % (International Classification D or E). Of patients with unilateral and bilateral disease, 35.2 % and 34.8 %, respectively, had extraocular disease at diagnosis; 10.7 % had metastatic disease at diagnosis. Enucleation was observed in 88.1 % and exenteration in 11.9 % of patients; 93.6 % patients were followed until 2012, and 22.9 % relapsed. Overall survival was 86.4 %. Conclusions: Most Rb diagnoses are still diagnosed in advanced stages of the disease, considerably reducing overall survival time and the rate of eye and vision preservation.

Treatment of retinoblastoma in Sub‐Saharan Africa: Experience of the paediatric oncology unit at Gabriel Toure Teaching Hospital and the Institute of African Tropical Ophthalmology, Bamako, Mali

“…Leukocoria was the most common presenting clinical feature of intraocular forms of Rb (n = 68; 77.2%), followed by strabismus (n = 9; 10.2%), consistent with medical literature . The unilateral (n = 50; 56.81%) and bilateral (n = 24; 27.3%) intraocular forms were predominant with majority being groups D and E, similar to the data from Brazil and China . The unilateral intra‐orbital forms accounted for 12.5% (n = 11) of Rb in our series.…”

“…29,30 The unilateral (n = 50; 56.81%) and bilateral (n = 24; 27.3%) intraocular forms were predominant with majority being groups D and E, similar to the data from Brazil and China. 31,32 The unilateral intra-orbital forms accounted for 12.5% (n = 11) of Rb in our series.…”

Experience with Retinoblastoma at a Tertiary Centre in Port Harcourt, Nigeria: Trends in Outcome

“…The median age at presentation in this study was 30 months, with 10% of the subjects presenting before their first birthday. Similar findings were reported in the Ilorin study, while it was at disparity with reports in India where median age at presentation was 24 months, with 42% of the study population presenting on or before 12 months of age, and in Southern Brazil with 35.7% presenting in that same age bracket [4,5,9]. It was not surprising that more subjects in these 2 series had bilateral disease, 52% in India, 32.9% in Brazil, compare to 26% in our series, as this type is known to usually present earlier in life [5,9].…”

“…In developed countries, RB is regarded as a rare tumour accounting for approximately 3% of all childhood malignancies and its current management has resulted in an improved survival to a rate of astounding 99% with more than 90% retaining normal visual acuity in at least one eye [8]. Whereas in developing nations, including african countries, where the majority of retinoblastoma cases live, it is considered one of the most frequent paediatric solid tumours with a higher incidence, and survival rate estimated at 40% [5,8,9]. This has been attributed to several factors, including lack of awareness, late presentation, parental cultural practices and traditional belief system, treatment abandonment/refusal of enucleation, absence of adequate healthcare facilities among others [3,8,[10][11][12].…”

Paediatric Retinoblastoma Presentation in a Regional Cancer Centre in Pakistan

“…Our institutional agenda is to propagate early detection and attempt optimal local tumour control with focal consolidative procedures such as laser photocoagulation, cryotherapy and local chemotherapy. We are working to introduce intravitreal and intra-arterial chemotherapy where possible [14] in early disease.…”