ABSTRACT
El sarcoma de Ewing óseo es un tumor poco frecuente, agresivo, que afecta principalmente a niños y adultos jóvenes. Existe ausencia de registros en nuestro país respecto de la prevalencia de esta enfermedad, los esquemas de tratamiento utilizados y sus resultados. El objetivo fue analizar, en un grupo de pacientes con sarcoma de Ewing óseo tratados con quimioterapia y cirugía de conservación de miembro, las tasas de supervivencia global, de recurrencia local y los factores de riesgo oncológicos. Se incluyó a 88 pacientes. La edad media de la serie fue de 14.5 años y el seguimiento promedio de 8.8 años. La tasa de supervivencia global fue de 79.5% a los 2 años, de 69% a 5 años y de 64% a 10 años. Los factores pronósticos negativos asociados a menor supervivencia fueron: mala respuesta a la quimioterapia, edad ≥ de 16 años, localización central, y recurrencia local. En el análisis multivariable únicamente la respuesta a la quimioterapia tuvo significancia estadística. La tasa libre de recurrencia local a 2 y 5 años fue del 87%. La mala respuesta a la quimioterapia fue el único factor significativo para la recurrencia local. Consideramos que la cirugía de conservación de miembro asociada a quimioterapia pre y postoperatoria debe ser el tratamiento para el sarcoma de Ewing óseo, alcanzando de esta manera una supervivencia global a 5 años del 69%. En nuestra serie, la respuesta a la quimioterapia ha sido el factor pronóstico más relevante para supervivencia y recurrencia local.
Ewing sarcoma of the bone is a rare, highly aggressive tumor that typically affects children and young adults. In Argentina, the lack of Ewing's sarcoma registries reflects in the absence of information regarding prevalence, treatment protocols and patient´s outcome. The purpose of this study was to analyze, in a group of patients diagnosed with Ewing sarcoma of the bone, treated with chemotherapy and limb-conserving surgery, their overall survival rate, local recurrence rate, and oncological risk factors. A retrospective research was conducted between 1990 and 2017. Eighty-eight patients with Ewing sarcoma of the bone matched the inclusion criteria. Median age was 14.5 years and median follow-up was 8.8 years. Overall survival rate was 79.5%, 69% and 64% at 2, 5 and 10 years respectively. Negative prognostic factors, associated with less survival rate after univariate analysis, were: bad response to chemotherapy (tumoral necrosis 0-89%), age > 16 years-old, central tumor localization and local recurrence. Gender and tumor size were not significant prognostic factors. After multivariate analysis, response to chemotherapy remained statistical significant. Local recurrence-free survival rate at 2 and 5 years was 87%. Tumor response to chemotherapy (0-89%) was the only significant factor for local recurrence. We consider that limb-salvage surgery, with neoadjuvant and adjuvant chemotherapy, are the mainstays of treatment for Ewing's sarcoma, with an overall survival rate, at 5 years, of 69%. In this population, response to chemotherapy is the most relevant prognostic factor, being associated with both local recurrence and overall survival.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Young Adult , Sarcoma, Ewing/mortality , Bone Neoplasms/mortality , Argentina/epidemiology , Sarcoma, Ewing/therapy , Time Factors , Bone Neoplasms/therapy , Logistic Models , Multivariate Analysis , Retrospective Studies , Risk Factors , Disease-Free Survival , Kaplan-Meier Estimate , Neoplasm Recurrence, LocalABSTRACT
PURPOSE: In order to avoid functional disability that may be caused by radical excision or amputation in extremity soft tissue sarcomas, authors employed limb-conserving surgery together with extemal radiation therapy plus interstitial brachytherapy. MATERIALS AND METHODS: From June 1995 to Febrary 1997, 10 extremity soft tissue sarcoma patients were treated with limb-conserving surgery and external radiation therapy plus interstitial brachytherapy. In six patients, whose histologic diagnoses were made at the time of surgery, wide or marginal excision and interstitial brachytherapy was done 4 weeks before postoperative external radiation therapy. In four patients whose histologic confinnations were done before definitive treatment, preoperative external radiation therapy was given 4 weeks before surgery and interstitial brachytherapy. The types of surgery were wide excision in five patients, and marginal excision in five patients. Gross or microscopic residual was left at the surgical resection margins in four patients. The brachytherapy dose ranged from 17.5 Gy to 24 Gy and external beam radiation did from 40 Gy to 45 Gy. RESULTS: With the median follow-up duration of 21.5 months(range: 13 to 29 months); one local recurrence, and three new distant metastases were observed. There were three patients with wound complications attributable to the current treatment regimen. CONCLUSION: Satisfactory local tumor control may be achievable with limb-conserving surgery and external radiation therapy plus brachytherapy in patients with extremity soft tissue sarcomas, while more caution should be used to avoid wound problems.