Page 145 - TD-3-4

Tumor Discovery Primarily enucleated RB patient’s analysis

The incidence rate is 1 in 16,000 – 18,000 live births, low-income countries report survival rates of 77% and 40%,
1
with approximately 9,000 new cases added each year respectively, with significantly higher rates of mortality
worldwide. In the United States, the number of new cases and metastasis. Most of these low- and lower-middle-
7
2
detected annually has remained steady at 200 – 300 over income countries are underdeveloped or developing
the past 40 years. In contrast, higher incidence rates have countries in Asia and Africa, where delayed presentation
3,4
been reported in Europe. The median age at diagnosis is complicates the management of RB cases. Factors such as
18 months globally, although it is 14 months in high-income delayed presentation, inadequate treatment facilities, lack
countries and 30.5 months in low-income countries. In of trained ocular oncologists and histopathologists, and
5
unilateral retinoblastoma, the median age of diagnosis was financial challenges contribute to higher child mortality
35 and 19.7 months in low- and high-income countries, rates in these regions.
respectively, where’s 22.9 and 8.1 months, respectively, in This study aimed to describe the pattern of HRFs
bilateral cases. 5
following primary enucleation in patients with RB as
RB presentation and outcomes vary by age at diagnosis well as the presentation, histopathological features, and
and national income level. Leukocoria is the most common survival outcomes of these patients in a lower-middle-
presenting sign, followed by strabismus; these early income country in Southeast Asia.
presentations are more typical in high-income countries.
In contrast, advanced presentations, such as proptosis, 2. Methods
orbital cellulitis, and red eye, are more frequent in low- 2.1. Study design
income countries. 5
This hospital-based retrospective study analyzed patients
The tumor is typically confined to the globe at early with RB who had undergone primary enucleation. Data
stages, supporting both ocular salvage and higher were collected from the Orbit, Oculoplasty, and Ocular
survival rates. Treatment modalities include enucleation, Oncology Department of Chittagong Eye Infirmary, a
chemotherapy (various routes), radiotherapy, and local tertiary eye care and retinoblastoma center in Bangladesh.
therapy. A multidisciplinary team approach, involving Data from January 2012 to December 2023 were included,
ophthalmologists (ocular oncologists, retina specialists, covering a 12-year period. A total of 96 children with RB
pediatric ophthalmologists, and/or oculoplastic who had undergone primary enucleation were classified as
surgeons), pediatric oncologists, radiation oncologists, Groups D and E. Among these, 78 patients were included
anesthesiologists, histopathologists, ocularists, counselors, in the study.
and social support groups, is the current standard for RB
treatment. In early cases, treatment may be limited to local 2.2. Tumor grading, staging, and HRFs
therapy and chemotherapy. However, in delayed cases, Tumors were classified according to the International
treatment options include enucleation or neoadjuvant Intraocular Retinoblastoma Classification (IIRC) for
chemotherapy, followed by secondary enucleation of the intraocular tumors and the International Retinoblastoma
worse eye, with or without radiotherapy. Some patients Staging System (IRSS) for extraocular tumors based on
may require adjuvant chemotherapy with or without data obtained from medical records, evaluation under
radiotherapy following primary enucleation. For all anesthesia (EUA), imaging, and histopathology reports.
9,10
enucleated cases, histopathology plays a crucial role in The IIRC categorizes tumors as follows:
guiding the management plan, making histopathological • Group A: Tumor ≤3 mm, ≥3 mm from the fovea, and
examination of enucleated eyes mandatory. The presence ≥1.5 mm from the optic disc, with no seeding.
of histopathological high-risk factors (HRFs) can provide • Group B: Tumor of any size or location (except
early indications of local recurrence, distant metastases, Group A criteria), without vitreous or subretinal
tumor progression, and overall prognosis. Key HRFs seeding, and with subretinal fluid ≤5 mm from the
associated with metastases include invasion of the choroid, tumor margin.
sclera, extrascleral space, and optic nerve (beyond the • Group C: Discrete tumor of any size or site with focal
lamina cribrosa, total optic nerve, or cut end). Expert vitreous or subretinal seeding and subretinal fluid up
6
histopathologists are thus integral to the multidisciplinary to one quadrant.
management team for RB. • Group D: Diffuse vitreous or subretinal seeding and/or
In high-income and upper-middle–income countries, extensive endophytic (tumor spread toward vitreous
the 3-year survival rates are approximately 99% and 91%, cavity) or exophytic disease (subretinal tumor spread),
respectively, with high ocular salvage rates and fewer with seeding exceeding Group C criteria and retinal
reported HRFs. In contrast, lower-middle-income and detachment of more than one quadrant.
7,8

Volume 3 Issue 4 (2024) 2 doi: 10.36922/td.4336