Background: In high income countries, retinoblastoma is curable in more than 95% of cases, whereas in low-income countries, mortality remains high, especially when the diagnosis is made late or the treatment is discontinued. The aim of this work was to determine the factors associated with adherence to the treatment of retinoblastoma in the Ivory Coast and the Democratic Republic of Congo (DRC). Procedure: A retroprospective cohort study was carried out. Data were collected from patient folders and follow-up records of parents. Results: A total of 175 children with retinoblastoma were registered from January 2013 to December 2015. Seventy-six children (43%) were 5 years old and above. Care costs were covered by families in 86.9% of cases. Chemotherapy refusal was recorded in 39 cases (22.3%), and enucleation refusal was recorded in 79 cases (45.1%). After 36 months of follow-up, we recorded 16.6% deaths, 27.4% treatment dropouts, and 18.3% loss to follow-up after treatment. The commonest cause for enucleation refusal was fear of infirmity, while chemotherapy refusal and absconding treatment were due to financial constraints. Conclusion: Poor adherence to retinoblastoma management was due to financial constraints, and a lack of knowledge of the disease and its treatment. Family psychosocial support is needed to improve this condition.

Introduction: The risk of relapse in retinoblastoma is currently determined by the presence of high-risk histopathologic factors in the enucleated eye. However, the probability of developing metastatic disease is heterogeneous among these patients. Evaluating a biological marker to identify high-risk patients could be useful in clinical setting. This study aims to evaluate whether the expression of TFF1, a surrogate for subtype 2 retinoblastoma, is a prognostic marker for relapse and death. Methods: This multicenter cohort study included 273 patients, 48 of whom had extraocular disease. Immunohistochemical staining were performed for CRX, ARR3, TFF1 and Ki67. Tumors were classified as histological subtype 1 (HS1) if they had low or no expression of TFF1 (quick score (QS) ≤ 50) and as histological subtype 2 (HS2) if they expressed TFF1 diffusely (QS > 50). We studied the association between HS classification and outcome. Results: Of 273 patients, 35.9% were classified as HS1, 59.3% as HS2 and 4.8% were not evaluable. In multivariate analysis, patients with HS2 tumors had a higher probability of relapse and death than those with HS1 ( P < 0.0001 and P = 0.00020, respectively). We identified a higher-risk subgroup among HS2 tumors, presenting non-mutually exclusive expression of ARR3 and TFF1 and had an increased risk of relapse and death compared to tumors that displayed mutually exclusive expression ( P = 0.012 and P = 0.027, respectively). Conclusions: Expression of TFF1, especially when it is not-mutually exclusive with ARR3, is an independent prognostic marker of poor outcome in retinoblastoma.

Background: In developed countries, retinoblastoma is curable in more than 95% of cases, whereas in low-income countries, mortality remains high, especially when the diagnosis is made late or the treatment is discontinued. The aim of this work was to determine the factors associated with adherence to the treatment of retinoblastoma in the Ivory Coast and the Democratic Republic of Congo (DRC). Procedure: A retrospective cohort study was carried out. Data were collected from patient folders and follow-up records of parents. Results: A total of 175 children with retinoblastoma were registered from January 2013 to December 2015. Seventy-six children (43%) were 5 years old and above. Care costs were covered by families in 86.9% of cases. Chemotherapy refusal was recorded in 39 cases (22.3%), and enucleation refusal was recorded in 79 cases (45.1%). After 36 months of follow-up, we recorded 16.6% deaths, 27.4% treatment dropouts, and 18.3% loss to follow-up after treatment. The commonest cause for enucleation refusal was fear of infirmity, while chemotherapy refusal and absconding treatment were due to financial constraints. Conclusion: Poor adherence to retinoblastoma management was due to financial constraints, and a lack of knowledge of the disease and its treatment. Family psychosocial support is needed to improve this condition.

Introduction: Advanced intraocular retinoblastoma can be cured by enucleation, but spread of retinoblastoma cells beyond natural limits of the eye may lead to metastatic disease which is related to a high mortality. Adjuvant therapy after enucleation has been shown to prevent metastasis in children with risk factors for extraocular retinoblastoma. However, histological criteria and adjuvant treatment regimens vary and there is no unifying consensus on the optimal choice of treatment. Method: Data on guidelines for adjuvant treatment in European retinoblastoma referral centres were collected in an online survey among all members of the European Retinoblastoma group (EURbG) network. Extended information were gathered via personal Email communication. Results: Data were collected from 26 centres in 17 countries. Guidelines for adjuvant treatment were in place at 92.3% of retinoblastoma centres. There was a consensus on indication for and intensity of adjuvant treatment among more than 80% of all centres. The majority of centres use no adjuvant treatment for isolated focal choroidal invasion or prelaminar optic nerve invasion. Patients with massive choroidal invasion or postlaminar optic nerve invasion receive adjuvant chemotherapy, while microscopic invasion of the resection margin of the optic nerve or extension through the sclera are treated with combined chemo- and radiotherapy. Conclusion: Indications and adjuvant treatment regimens in European retinoblastoma referral centres are similar but not uniform. Further biomarkers in addition to histopathological risk factors could improve treatment stratification. The high consensus in European centres is an excellent foundation for a common European study with prospective validation of new biomarkers.