ABSTRACT
BACKGROUND: The aim of this study is to evaluate the relationship between the latency to diagnosis (LD) and the time to completion of chemotherapy (TCC) with clinical outcomes in children with osteosarcoma. METHODS: We performed a retrospective analysis of all patients who received treatment for osteosarcoma in two tertiary centers in Peru from 2008 to 2015. All causes of delayed LD or TCC were evaluated. Overall survival (OS) and event-free-survival (EFS) were estimated and compared according to LD, TCC, and established clinical prognostic factors. RESULTS: One hundred and thirteen patients were included in the study. The median LD was 13.5 weeks (interquartile range, 10-18.5 weeks). No association was observed among clinical stage, tumor size, and LD. Delayed LD was not associated with a worse clinical outcome. Multivariate analysis confirmed that OS and EFS were significantly worse in cases of a delayed TCC (≥4 weeks), with hazard ratios of 2.70 (1.11-6.76, P = 0.003) and 1.13 (1.00-1.26, P = 0.016), respectively. Most delays in TCC (85%) were due to extramedical reasons (e.g., lack of available hospital beds). CONCLUSION: The LD did not seem to influence the EFS and OS in pediatric patients with osteosarcoma. However, a delay in TCC from any cause is independently associated with poor outcome in pediatric patients with osteosarcoma. Based on these results, further efforts may be needed to avoid treatment delays in patients with osteosarcoma in middle-income countries.
Subject(s)
Bone Neoplasms , Osteosarcoma , Adolescent , Bone Neoplasms/diagnosis , Bone Neoplasms/mortality , Bone Neoplasms/therapy , Child , Child, Preschool , Disease-Free Survival , Female , Humans , Male , Osteosarcoma/diagnosis , Osteosarcoma/mortality , Osteosarcoma/therapy , Retrospective Studies , Survival RateABSTRACT
Objetivo: Describir las características clínicas y epidemiológicas, y determinar los factores pronósticos, sobrevida libre de evento (SLE) y sobrevida global (SG) de los pacientes con tumores de la familia del Sarcoma de Ewing (TFSE). Materiales y métodos: Estudio retrospectivo llevado a cabo en pacientes menores de 18 años con TFSE, atendidos en la Unidad de Oncología Pediátrica y del Adolescente del Hospital Edgardo Rebagliati entre 2006 y 2016. El análisis descriptivo se realizó mediante distribución de frecuencias. Para el análisis de SLE y SG se utilizaron las curvas de Kaplan-Meier. Se efectuó un análisis univariado y multivariado según modelo de regresión de Cox para variables demográficas, clínicas y quirúrgicas, y factores pronósticos. La medida de fuerza de asociación se expresó en hazard ratio (HR) e intervalo de confianza al 95% (IC 95%), y se consideró p<0.05 para diferencias significativas. Resultados: Se presentaron 29 casos de TFSE. La mediana de edad fue de 9 años (rango 2-17), el 55% fueron varones. La localización más frecuente fue la pelvis (31%). El 59% presentaron metástasis al diagnóstico. La SLE a 3 años en TFSE localizados fue del 40.4% (±14.4 EE) y con metástasis, 14.6% (±12.2, EE). La SG a 3 años en TFSE localizados fue del 53.9% (±17.8 EE) y en enfermedad metastásica, 15.1 % (±9.7, EE). El tamaño tumoral ≥5cm (HR 14.84, p=0.01) y la presencia de metástasis al debut (HR 3.23, p=0.01) fueron factores pronósticos independientes de peor SG. No hubo diferencia significativa en relación con el pronóstico según el sexo, edad, tipo histológico, compromiso de los bordes quirúrgicos o localización del tumor. Conclusiones: Los TFSE son altamente agresivos. Los factores pronósticos que contribuyen a una menor SLE y SG son la presencia de metástasis al debut de la enfermedad y un tamaño tumoral ≥5cm.
Objective: To describe the clinical and epidemiological characteristics, and to determine prognostic factors, event-free survival (EFS) and overall survival (OS) of patients with Ewing sarcoma family tumors (ESFT). Materials and methods: A retrospective study conducted in patients under 18 years of age with ESFT, treated at the Unit of Pediatric and Adolescent Oncology of the Hospital Edgardo Rebagliati between January 2006 and June 2016. The descriptive analysis was performed by frequency distribution. Kaplan-Meier curves were used for EFS and OS analysis. Univariate and multivariate analyses were performed according to the Cox regression model for demographic, clinical and surgical variables, and prognostic factors. The measure of strength of association was expressed in hazard ratio (HR) and 95% confidence interval (95% CI), and p<0.05 was considered for significant differences. Results: There were 29 cases of ESFT. The median age was 9 years (range 2-17), 55% were males. The most frequent location was the pelvis (31%). Fifty-nine percent (59%) had metastasis at diagnosis. The 3-year EFS in localized ESTF was 40.4% (± 14.4 EE) and with metastasis was 14.6% (± 12.2, EE). The 3-year OS in localized ESTF was 53.9% (± 17.8 EE) and with metastatic disease was 15.1% (± 9.7, EE). The tumor size ≥5cm (HR 14.84, p=0.01) and the presence of metastasis at the onset of the disease (HR 3.23, p=0.01) were independent prognostic factors of worse OS. There was no significant difference in prognosis regarding the gender, age, histological type, involvement of surgical borders or location of the tumor. Conclusions: ESFT are highly aggressive. The prognostic factors that contribute to a lower EFS and OS are the presence of metastasis at the onset of the disease and a tumor size ≥5cm.
ABSTRACT
BACKGROUND: The aim of this retrospective study was to define clinical and pathological features and prognostic factors among children and adolescents diagnosed with high-grade osteosarcoma of the extremities. METHODS: A total of 73 patients younger than 18 years diagnosed with primary osteosarcoma of the extremities between January 1998 and December 2013 were retrospectively evaluated. Prognostic factors, such as age, gender, primary tumor site, alkaline phosphatase and lactate dehydrogenase levels, metastatic disease, pathological fracture, histological response, and surgery type, were analyzed to evaluate their effects on overall survival (OS) and event-free survival (EFS). RESULTS: At a median follow-up of 30 months (1.5-152), OS and EFS at 5 years were 64.5 ± 8.1 and 48.5 ± 8.7% for patients with localized disease; and 16.2 ± 7.9 and 14.4 ± 7.3% for patients with initial metastatic disease, respectively. In patients with localized disease, conservative surgery was performed on 22 of 46 patients (43.5%), and there was no significant difference in survival rates among patients who had conservative vs. radical surgery (p = 0.65). Although tumor size (>12 cm) was significant prognostic factor in univariate analysis; multivariate analysis identified elevated levels of alkaline phosphatase (p = 0.033) and poor response to neoadjuvant chemotherapy (p < 0.001) only as independent prognostic factors. Age, histological type, pathological fracture, and primary tumor site did not significantly affect prognosis. CONCLUSION: Initial elevated presence of alkaline phosphatase in serum and poor histological response after neoadjuvant chemotherapy were significant factors for unfavorable prognosis. It is necessary to optimize staging and treatment intensification to improve survival rates, especially among patients with metastasis at initial presentation.