Enucleated globes with advanced retinoblastoma: correlation of histopathological features and reclassification of tumors according to the 8th edition of the American Joint Committee on Cancer (AJCC)

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

“…There was no significant association between HRF and tumor differentiation, in line with Alkatan et al [4] and Kashyap et al [10] who demonstrated no statistically significant correlation between tumor differentiation and HRFs, except for massive choroidal invasion [5]. This insignificant association most likely relates to the tumor genome; genomic and gene expression changes are not associated with high-risk pathological features rather affect tumor differentiation [13].…”

“…This is in line with studies by Wilson et al [36] and Kashyap et al [31] that found approximately 80% cases and up to 95% cases, respectively, linked to the age at diagnosis of ≥24 months. Alkatan et al [4] reported a lower incidence (52%); however, these results also showed that poorly differentiated tumors are more common than moderately/well-differentiated tumors. In the present study, the age at diagnosis of >2 years was significantly associated with poorly and undifferentiated tumors, whereas previous studies found different results in terms of the relationship between tumor cell differentiation and RB outcomes.…”

“…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.…”

“…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. Several studies have investigated the association between age, tumor differentiation, and HRFs, reporting that less tumor differentiation is a relative risk of massive choroidal invasion, and moderately differentiated tumors occurring at advanced ages are more frequently associated with HRFs, thus reflecting a delayed diagnosis [4,[12][13][14]. HRFs of RB may lead to metastasis if not treated with systemic adjuvant chemotherapy [4,15,16].…”

“…Several studies have investigated the association between age, tumor differentiation, and HRFs, reporting that less tumor differentiation is a relative risk of massive choroidal invasion, and moderately differentiated tumors occurring at advanced ages are more frequently associated with HRFs, thus reflecting a delayed diagnosis [4,[12][13][14]. HRFs of RB may lead to metastasis if not treated with systemic adjuvant chemotherapy [4,15,16]. Nonetheless, there is some controversy regarding the definition of high-risk histopathologic features as well as the most effective chemotherapeutic protocol to prevent unnecessary chemotherapy use, especially for those who are at low risk for relapse.…”