Pathological Risk Factor Profile for Enucleated Retinoblastoma at Sanglah General Hospital

Abstract

Retinoblastoma is a primary intraocular tumor on childhood. It has a good prognosis with early detection. Aim of this study is to know Pathological Risk Factors (PRF) profile of enucleated retinoblastoma at Sanglah General Hospital. This study is an observational retrospective study based on patient’s medical record and pathological examination. Twenty patients were included in this study, 50% were female and 50% male within age ranging from 7 month – 12 years old. Unilateral retinoblastoma was found in 18 patients (90.5%), bilateral retinoblastoma was found in 2 patients (9.5%). Majority PRF profile found that cancer cell invasions was 60% choroid infiltration and 45% of anterior segment invasion, 60% of optic nerve infiltration, and 50.1% scleral invasion. Tumor cell aggregation showed 61.9% poorly differentiated malignant cells. None of the patients had family history of retinoblastoma. This study revealed optic nerve and choroid invasion were the major PRF found in retinoblastoma, but there is a significant association between scleral infiltration and tumor cell differentiation (p = 0.025). There was no statistical difference of PRF based on age, gender, and laterality.

High-Risk Histopathologic Features of Retinoblastoma Treated at a Tertiary Hospital in West Java, Indonesia

“…Our study reported most of RB diagnosed at 25-36 months (32.2%). In developed countries, such as USA and Britain, RB is diagnosed early in childhood at an average age of 18 months, while the diagnosis occurs later in developing countries [2,24], such as in Indonesia [5,25]. Sixty-six patients were female, consistent with the study of Singh et al [26] and Owoeye et al [27] who described a higher incidence of RB in females than males.…”

“…According to the laterality, unilateral RB is more common than bilateral RB [2,24,30], as observed in this study with more unilateral cases than bilateral cases. Another study conducted in Indonesia by Yuliawati and Ekawati [5] also showed a similar result.…”

“…Clinical features related to tumor genomic and transcriptomic profiles can facilitate a better understanding of disease progression. Thus, well-differentiated tumors have more cone signatures than other tumor differentiations and are more common in younger patients [5,10]. Tumors with a reduced cone signature generally have a large volume, are often found in larger eyes, and are located peripherally or throughout the retina, representing an advanced stage disease.…”

“…Retinoblastoma (RB) is the most common solid neoplasm in childhood, representing 3% of all pediatric cancers [1,2]. In Indonesia, RB is generally found in advanced conditions or groups D and E based on the International Intraocular Retinoblastoma Classification (IIRC) [3][4][5][6]. Generally, RB is due to the lack of awareness among the population, difficult access to health care, lack of medical staff, limited histopathological examination service, and the absence of standardized surgical and histopathological examination protocols.…”

“…After enucleation, a histopathological examination to identify high-risk histopathological features (HRFs) and tumor differentiation is performed [5,9]. Previous studies have shown that HRFs are more common in developing countries than developed countries [6,7,10,11]; however, the role of tumor differentiation in RB outcomes has not been well investigated.…”