Long-term follow-up of retinoblastoma survivors: Experience from India

Abstract

Background:Retinoblastoma (Rb) is the most common primary intraocular tumor of infancy and childhood. Survivors’ ocular and visual problems and increased risk for subsequent malignancy are well documented, but data on long-term health status of Rb survivors are limited, this being particularly true for India.Methodology:Children who had completed treatment for Rb at least 2 years ago before and were under follow-up at the after cancer treatment clinic were evaluated.Results:In our series of 213 patients, the median age was 29 months, there was a male preponderance, and majority had unilateral disease. Enucleation was done in almost three-fourth and 3% underwent bilateral enucleation. Majority of the patients received chemotherapy, and few received radiation. Growth was affected in about one-third and majority were those who had received radiation. Diminished vision was noticed in about one-sixth. Orbital hypoplasia and contracted socket were seen in 14.1% cases. 2.7% were hearing impaired. About one-sixth had a global intelligence delay. Second neoplasms were seen in 0.01%. No other abnormalities were seen.Conclusions:Common late effects in our Rb survivors include diminished vision in the salvage eye, intellectual disability, and contracted socket; there is a need for timely institution of prosthesis to avoid late effects such as hypoplasia, contracted sockets, and better cosmesis and enhanced self-esteem. Second neoplasm is a concern. Lifelong follow-up and counseling of a healthy lifestyle are needed for Rb survivors.

Childhood cancer survivorship and late effects: The landscape in India in 2020

“…Despite the above limitations, several important observations can be made. These studies confirmed some of the well‐documented issues in childhood cancer survivors including the previously reported detrimental effects of cranial radiation on neurocognition 10‐13 and neuroendocrine axis, 12 of neck radiation on thyroid function, 8,11 and of alkylating agents on fertility 11,13,25,26 . Contrary to published literature, 29 a single study from India showed that bone mineral density in leukemia survivors was similar to that of controls 23 .…”

“…A summary of results of 21 studies 8‐28 is tabulated (Supporting Information Table 1) and categorized by organ systems and late effects as used in the Children's Oncology Group Survivorship Guidelines 29 . All except one 8 of these studies have been published in the last decade, highlighting the recent expansion in work in this area in India.…”

“…A median follow‐up was 4.5 years (range, >1‐12 years). Most studies lacked controls, 8‐15,18‐22,24‐28 which is important to quantify the increased risk of the problem as compared with the general population. Importantly, with few exceptions, 10,12,25 most studies had not examined dose‐effect relationship of exposure or the effect of combined modality of treatment.…”

Metabolite systems profiling identifies exploitable weaknesses in retinoblastoma

“…Additionally, patients with germ line mutation are prone to secondary cancers compared to the general population [8]. However, both enucleation and chemotherapy have severe side effects, including intellectual and developmental disabilities [11][12][13]. Currently, intravitreal chemotherapy with melphalan and intra-arterial chemotherapy with carboplatin are commonly practiced to save the vision of these children.…”

“…If these measures fail, enucleation is done [9,10]. However, both enucleation and chemotherapy have severe side effects, including intellectual and developmental disabilities [11][12][13]. Recently, metabolomics on RB samples identified a number of metabolites (majorly from amino acid and lipid metabolism), concentrations of which significantly differed from healthy tissue [14].…”