Epidemiology and clinical features of retinoblastoma

Abstract

Introduction: Retinoblastoma (RB) is a prototype of heritable cancers. It is more common in the lower socioeconomic strata. Delayed presentation significantly reduces the overall outcome. We have analyzed the epidemiological and clinical data of children who were diagnosed with RB between the years 2009 and 2014. Aim: RB being a disease of the poor, delayed presentation is common due to lack of awareness. We have analyzed the epidemiological profile of our patients and tried to establish the link between delayed presentation and the presence of high-risk features. High-risk features are associated with higher chance of metastasis and poor rates of vision salvage in RB. Methodology: Data were collected in a retrospective manner from the patient case files retrieved from the Medical Records Department, Kidwai cancer Institute. The data were analyzed using Excel and SPSS software (IBM Corp. released 2016, IBM SPSS statistics software for Mac OS, version 24, IBM Corp., Armonk, NY). Results: A total of 53 patients were diagnosed with RB in the years 2009–2014. There was a male predominance with 1.2:1 incidence. Bilateral RB was present in 21 cases. The mean age of children with bilateral RB was 2.1 years, against 1.5 years in unilateral cases. High-risk features such as optic nerve invasion, choroidal invasion, intracranial extension, and orbital involvement were found in 12, 6, 5, and 5 eyes, respectively. Bone marrow involvement was detected in 5% and lung metastasis in 2%. Intracranial involvement was found in 10.4% and cerebrospinal fluid positivity in 15%. Children with high-risk features had a significant delay in presentation in comparison to those without high-risk features ( P = 0.035). Conclusion: Incidence of metastatic disease and delayed presentation is still high in developing countries. Routine eye examination during vaccination visits can ensure early diagnosis and appropriate referral in many of these children.

Clinicoepidemiological Profile and Treatment Outcomes in Children with Retinoblastoma: Experience from a Cancer Care Center in Northeast India

“…11 The median age at presentation in our study was found to be 14 months, which is lower than 3.5 years reported by the study from Sahu et al 12 and 30 months reported in the study from Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. 13 and is similar to the study by Padma et al 14 Our study found slightly male predominance with a male to female ratio of 1.2:1, which was similar to that reported from other Indian studies. 12 14 The median duration of illness in our study group is 1.5 months, with a family history of RB and of other malignancy found is 0.5 and 4%, respectively.…”

“…13 and is similar to the study by Padma et al 14 Our study found slightly male predominance with a male to female ratio of 1.2:1, which was similar to that reported from other Indian studies. 12 14 The median duration of illness in our study group is 1.5 months, with a family history of RB and of other malignancy found is 0.5 and 4%, respectively. Study from Tata Memorial Hospital, Mumbai, Maharashtra, India, reported a longer duration of illness (i.e., 8 months) before presentation and similar family history of malignancy, as compared with our study.…”

“…Most of the children in our study presented at an advance stage (stage D—21% and stage E–76%) as compared with 78% reported in study from PGIMER, Chandigarh, India. 13 Extraocular disease at presentation was found in 29% of children similar to 27% reported from PGIMER, Chandigarh, India 13 but was found to be less than as compared with 57 to 58% reported in study by Sahu et al and Padma et al 12 13 14 However, these figures are in contrast to the Western world, 16 where the incidence of extraocular disease has been reported to be less than 5%. Therefore, a major challenge in our country is the implementation of an early detection program to minimize the progression of RB to advanced stages and allow for the treatment at earlier stages of disease and have better treatment outcomes.…”

“…As compared with study by Padma et al, where only 55% children agreed to and underwent treatment, in our study 82% children underwent treatment. 14 One more possible reason of declining treatment by parents is the counseling regarding enucleation for advanced disease. A study from Malaysia 17 reported that most families refuse treatment upon counseling in favor of enucleation.…”

Clinical Features, Treatment, and Outcomes of Retinoblastoma in China

“…Refusal for treatment including enucleation and treatment abandonment in China was low, a common occurrence in other Asian coutries. 21 22 23 However, some articles used treatment refusal and abandonment as exclusion criteria which may explain the low numbers.…”