Maria Carmen Affinita

and 17 more

not-yet-known not-yet-known not-yet-known unknown Background: Timely diagnosis is critical in pediatric oncology to optimize treatment outcomes. Diagnostic delays may impact tumor extension and prognosis, necessitating analysis of diagnostic intervals across different neoplasms. Methods: We analyzed data from 749 pediatric patients diagnosed with rhabdomyosarcoma between 1996 and 2016. Diagnostic interval (DI) was defined as days from symptom onset to diagnosis, and treatment interval (TI) from symptom onset to treatment initiation. Factors influencing DI and TI were collected, including patient age, histology, tumor characteristics, and protocol of treatment. Survival outcomes were assessed using Kaplan-Meier analysis. Results: Median DI was 32 days, decreasing insignificantly from 1996-2004 to 2005-2016. Longer DI was associated with metastatic disease (p=0.0021). The proportion of patients diagnosed within one month increased over time, but remained lower for metastatic cases. Median TI was 48 days, unchanged over time. Longer TI correlated with larger tumors (p<0.0001). Adolescents had prolonged DI (>2 months) more frequently. The quantile regression models showed that on univariate analysis DI was associated with age at diagnosis, unfavourable histology and metastatic diaeses, but not confirmed in multivariate Five-year event-free survival (EFS) and overall survival (OS) were 59.7% and 69.3%, respectively. Conclusions: This study evaluated the role of timely diagnosis and treatment initiation in pediatric patients with rhabdomyosarcoma . Our data highlights that DI and TI are crucial in adolescents and often longer in metastatic patients. Future efforts should focus on streamlining access to diagnostic facilities and improving processes to ensure timely interventions, especially for patients presenting with more advanced disease.
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.