Clinical features and outcomes in patients with extraskeletal ewing sarcoma

Abstract

BACKGROUND: Ewing sarcoma can arise in either bone or soft tissue. The purpose of this study was to investigate whether patient characteristics, treatment strategies, and outcomes differ between skeletal Ewing sarcoma and extraskeletal Ewing sarcoma (EES). METHODS: Patients <40 years of age with Ewing sarcoma or peripheral primitive neuroectodermal tumor reported to the United States Surveillance, Epidemiology, and End Results Program database from 1973 to 2007 were evaluated based on skeletal (n ¼ 1519) versus extraskeletal (n ¼ 683) site of origin. Patient characteristics were compared using Fisher exact tests. Overall survival was estimated via the Kaplan-Meier method and compared using log-rank tests and Cox proportional hazard models. RESULTS: Patients with EES had a higher mean age (19.5 vs 16.3 years; P < .001) and were less likely to be male (53.4% vs 63.3%; P < .001) or white (84.8% vs 92.5%; P < .001) compared with patients with skeletal tumors. Extraskeletal tumors were more likely to arise in axial locations (72.9% vs 54.2%; P ¼ .001) but were less likely to arise specifically in the pelvis (19.8% vs 26.6%; P < .001). Metastatic status or tumor size did not differ by group. Five-year overall survival was superior for localized EES compared with localized skeletal tumors (69.7% vs 62.6%; P ¼ .02). The hazard ratio for death in patients with localized skeletal tumors compared with localized EES was 2.36 (95% confidence interval, 1.61-3.44) beyond 24 months from initial diagnosis. CONCLUSIONS: Patient characteristics and outcomes differ among patients with EES compared with patients with skeletal Ewing sarcoma. These findings may have important implications for patient care. Cancer 2011;117:3027-

Treatment and Outcomes of Ewing Sarcoma in Turkish Adults: A Single Centre Experience

“…In our study, the median age was 27. Only 3.8% of patients (n=1) were older than 40 year, and 19% of the patients (n=5) were older than 30 years, which correlated with the center's other observations (Cotterill et al, 2000;Applebaum et al, 2011;Collier et al, 2011). Thus, many authors recommend that the clinician first eliminate other small round cell tumors such as small cell carcinoma and large cell lymphoma in patients older than 30 years (Iwamoto 2007).…”

“…The differences in tumor characteristics and clinical outcomes between skeletal and extraskeletal ES suggest that they have subtle biological differences. One of the causes of these differences may arise from the tumor's microenvironment (Applebaum et al, 2011). Another cause of the different clinical outcome is that it is more feasible to apply more effective local therapy in extremity primary ES.…”

Retrotracheal Extraskeletal Ewing’s Sarcoma: Case Report and Discussion on Airway Management

“…The most common translocation is gene EWSR1 on chromosome 22q1. This can be identified using fluorescent in situ hybridization or reverse transcription polymerase chain reaction (8).…”

Factores pronósticos y sobrevida en pacientes menores de 18 años con tumores de la familia del sarcoma de Ewing: experiencia de 10 años

“…Si bien algunos estudios mencionan que la ubicación extraósea de los TFSE tiene mejor sobrevida global que los TFSE óseos (17) , esto no se replicó en nuestro estudio, en donde la SLE y la SG no tuvieron diferencias estadísticamente significativas ni el análisis univariado ni el multivariado. Asimismo pudimos observar que en nuestro estudio la ubicación pélvica del tumor no se relacionó con una menor SLE o SG, a diferencia de lo publicado por Gupta et al (18) quienes reportaron una SLE y SG a los 3 años menor para aquellos pacientes con enfermedad pélvica (p=0.018) y de lo publicado por el Intergroup Ewing's Sarcoma Study (IESS) que reportó que los pacientes con tumores pélvicos tenía menores índices de sobrevida comparado con aquellos que tenían lesiones en extremidades (19) (Tabla 2 y 3).…”