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Tumor Discovery Primarily enucleated RB patient’s analysis

For unilateral RB, enucleation is often the preferred optic nerve invasion (38.4%, n = 30) was the second
treatment in many underdeveloped and developing most common HRF (Figure 4A and B). PLONI without
countries across Africa, Asia, and Latin America, where transection occurred in 26.9% (n = 21) of cases, and PLONI
alternative treatment options are limited. According to the with transection occurred in 11.5% (n = 9). Higher rates
Global Retinoblastoma Outcome Study, enucleation rates than ours have been reported in middle-income countries,
are high, with 73.6% in low-income countries and 67.1% in ranging from 39.6% to 49%. 8,33
lower-middle-income countries. This study also indicated A comparative study of 331 Indian and 193
7
that this rate in higher-income countries is 59.7%. Reddy American children showed that Indian children had
et al. reported that in Southeast Asia, enucleation is a five-fold greater risk of optic nerve invasion and a
25
the primary treatment for Group D and E unilateral RB. three-fold greater risk of massive choroidal invasion
Following enucleation, it is essential to send the enucleated
eye for histopathological evaluation, and the optic nerve
length should be >10 mm. Research suggests that a 10 –
15-mm long section of the optic nerve is curative in 85 –
90% of non-heritable unilateral cases without extraocular
involvement. 26
Histopathological analysis plays a crucial role in
identifying HRFs, as the subsequent treatment plan,
metastasis risk, and survival outcomes depend on HRF
presence. Older presentation age (>24 months) is associated
with HRFs. 8,26,27 In the present study, the median age at
presentation was 33 months, with 62% (n = 48) of patients
above 24 months. Among 78 patients who underwent
primary enucleation, 84.61% (n = 66) patients were HRF-
positive, supporting findings from previous studies. 8,26,27 Figure 3. Massive choroidal invasion >3 mm, observed in eosin and
hematoxylin (H&E) stain at 20× magnification. Scale bar: 200 µm
In the present study, a mixed endophytic and exophytic
growth pattern was observed in 36% of cases, followed A
by an exophytic pattern in 30%. Tumor differentiation
revealed a higher prevalence of poorly differentiated
tumors (44.9%, n = 35). Asian studies also indicate a trend
toward poorly differentiated tumors in older patients (over
24 months). 8,28
Delayed presentation contributed to multiple HRFs in
37.6% (n = 27) of our patients, aligning with rates reported
8,29
by Kaliki et al. (38%) and Yaqoob et al. (35.2%). Most
patients had massive choroidal invasion (41%, n = 32;
Figure 3), which is documented in the literature with B
prevalence ranging from 15.2% to 62%. Recent studies
26
have suggested that focal choroidal invasion if associated
with optic nerve invasion (pre-laminar and laminar),
is also a risk factor. Cases involving both choroidal and
optic nerve invasion have a poorer prognosis and higher
recurrence risk, necessitating careful adjuvant therapy. In
30
our study, 20.6% (n = 16) of patients exhibited both focal
choroidal and pre-laminar or laminar optic nerve invasion.

Survival and metastasis risk are significantly impacted
by optic nerve invasion, especially post-laminar optic
nerve invasion (PLONI) and optic nerve transection
involvement. Metastasis rates for PLONI range from 12%
to 42% and those for optic nerve transection involvement Figure 4. Optic nerve invasion by tumor. (A) Post-laminar invasion, Scale
range from 41% to 78%. 31,32 In this study, retrolaminar bar: 200 µm; (B) Optic nerve transection involved with tumor

Volume 3 Issue 4 (2024) 7 doi: 10.36922/td.4336