Page 149 - TD-3-4

Tumor Discovery Primarily enucleated RB patient’s analysis

the Global Retinoblastoma Presentation Study, the median our patients required bilateral enucleation after initially
age of presentation in unilateral cases was 27.1 months, presenting unilaterally, subsequently returning after a year
whereas that in bilateral cases was 12.3 months. The with advanced RB in the previously healthy eye. The family
5
median age of unilateral RB presentation in lower-middle- refused neoadjuvant chemotherapy, and enucleation was
income countries in the same study was 29.1 months. Our performed to save the child’s life.
country, a lower-middle-income country in Southeast Asia, The time from symptom onset to diagnosis is critical for
demonstrated a higher median age of presentation than RB outcomes. A symptom duration of 6 months or more
that mentioned in the above study. The age of presentation correlates with poorer outcomes. In the present study, the
may vary based on geographical location. A recent study mean symptom duration was 5.89 ± 4.30 months. A recent
5
on RB presentation across Asia reported a median age study reported an average lag time of 5 months for Asian
of 25 months, although it did not differentiate between children, 8 months in Southeast Asia, and 15 months
unilateral and bilateral cases. This study also found that in Central Asia. In sub-Saharan Africa, this period is
16
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the mean age was higher in South Asian countries than 8.5 months. A prolonged lag time results in more advanced
22
in other Asian regions. In the present study, the mean tumors, increasing enucleation rates. Lag time exceeding
16
age of presentation was 34.56 ± 19.90 months, whereas in 1 year is associated with a higher risk of extraocular
Southeast Asia, the median age was 30 months. A study invasion and increased mortality. Factors contributing
17
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from India showed a mean age range of 29 to 33 months, to longer lag times include poverty, reliance on traditional
18
whereas two separate studies from Pakistan reported medicine, limited awareness of treatment options, lack of
means of 36 ± 28 months and 42 ± 20 months. In the awareness among local pediatricians and physicians, and
present study, 8% of children were under 1 year of age, 30% geographic distance from treatment facilities. In our study,
were between 1 and 2 years, and 61% were above 2 years; we identified poverty, belief in traditional medicine, and
this distribution was similar to an Indian study where 38% lack of treatment facilities and physician awareness as the
of children were under 2 years and 62% were above 2 years main factors causing delays.
of age. 19
In the present study, 93.7% (n = 73) of patients
Leukocoria was the most common presenting symptom presented with advanced RB in Group E whereas only
in our study (70.5%), followed by red eye (20.5%). However, 7% presented with advanced RB in Group D. A review
global studies and most other studies have reported squint of 2,697 Indian patients with RB showed that 73 – 78%
as the second most common presentation. In a Chinese presented with advanced RB (Groups D and E). 19,20,24
5,15
study, leukocoria was reported in 67% of cases, followed by Another study on 700 Chinese children undergoing
red eye in 13%. An Eastern Indian study noted proptosis primary enucleation showed that 60% of patients in
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as the second most common sign after leukocoria, whereas Group E and 32% in Group D presented with advanced
squint was most frequent in other parts of India. 15,20 In RB. Among 78 study patients, 58% were in Stage I,
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the present study, late-stage symptoms, such as proptosis, 33% in Stage II, and 5% in Stage III. Accurate diagnosis
were observed in 6.4% of cases, and a painful blind eye was and treatment planning for RB necessitate grouping and
observed in 3.8% of cases. High-income countries, such staging, which requires EUA before initiating treatment.
as the United States and South Korea, have much lower Although RB is primarily diagnosed clinically, additional
rates of proptosis (0.5% and 1.4%, respectively), whereas imaging such as ultrasonography (B-scan), computed
this rate is significantly higher in lower-middle-income tomography (CT), and magnetic resonance (MRI)
countries, such as Nigeria (44.2%), Pakistan (52.8%), and may be necessary for unclear cases and to assess tumor
Thailand (26.7%). 18 extension. In this study, all patients underwent EUA and
B-scans; most had CT scans, while only a few had MRIs,
Our study observed a male predominance in sex
distribution (55.1% male, n = 43; 44.9% female, n = 35), primarily due to constraints. As CT scans present a risk
of radiation exposure, which is particularly concerning for
with a male-to-female ratio of 1.4:1, ; this finding was in hereditary RB cases, MRI is generally preferred. However,
1
line with the 1.52:1 ratio observed in India and 1.8:1 noted the high cost of MRI made it inaccessible for most of our
in Asian children by the Global Retinoblastoma Study patients. The Global Retinoblastoma Presentation Study
Group. 21
demonstrated a preference for imaging based on income
In this study, 10.25% (n = 8) of patients presented with level: lower-income countries performed CT scans in 69%
bilateral RB, with only 2.6% (n = 2) of patients reporting a of cases, MRIs in 16.5%, and both modalities in 17.1%. In
family history. While heritable RB often presents bilaterally, contrast, high-income countries performed CT scans in
approximately 12%–15% of unilateral cases may also have only 0.6% of cases, MRIs in 34.7%, and both imaging types
a heritable basis and can later manifest bilaterally. One of in 64.7%. 7
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Volume 3 Issue 4 (2024) 6 doi: 10.36922/td.4336