scholarly journals Extraskeletal Ewing sarcoma in children, adolescents, and young adults. An analysis of three prospective studies of the Cooperative Weichteilsarkomstudiengruppe (CWS)

2021 ◽  
Author(s):  
Ewa Koscielniak ◽  
Monika Sparber‐Sauer ◽  
Monika Scheer ◽  
Christian Vokuhl ◽  
Bernarda Kazanowska ◽  
...  
Author(s):  
Mohammadreza Mortazavi ◽  
Nancy Baxter ◽  
Sumit Gupta ◽  
Abha Gupta ◽  
Cindy Lau ◽  
...  

Abstract Background: Location of cancer care (LOC: pediatric versus adult center) impacts outcomes in adolescents and young adults (AYA) with some cancer types. Data on impact of LOC on survival in AYA with osteogenic sarcoma (OGS) and Ewing sarcoma (EWS) are limited. Objectives: To compare differences in demographics, disease/treatment characteristics, and survival in a population-based cohort of AYA with OGS or EWS treated at pediatric versus adult centers Methods: The IMPACT Cohort captured demographic, disease, and treatment data for all AYA (15-21 years old) diagnosed with OGS and EWS in Ontario, Canada between 1992-2012. Patients were linked to provincial administrative healthcare databases. Outcomes were compared between patients treated in pediatric versus adult centers using appropriate statistical methods. Results: 137 AYA were diagnosed with OGS (LOC: 47 pediatric, 90 adult) and 84 with EWS (LOC: 38 pediatric, 46 adult). AYA treated at pediatric centers were more likely to be enrolled in a clinical trial (OGS 55% vs 1%, [p<0.001]; EWS 53% vs 2%, [p<0.001]) and received higher cumulative chemotherapy doses. Five-year event-free survival (EFS ± Standard Error) in OGS and EWS were 47% ± 4 and 43% ± 5, respectively. In multivariable analysis, the impact of LOC (pediatric vs adult center) on EFS in OGS (adjusted hazard ratio [HR] 1.15, 95% CI 0.58-2.27, P=0.69) and EWS (adjusted HR 1.82, 95% CI 0.97-3.43, P=0.06) were not statistically significant. Conclusion: Outcomes did not differ by LOC suggesting that AYA with bone tumors can be treated at either pediatric or adult centers.


2014 ◽  
Vol 32 (15_suppl) ◽  
pp. 10530-10530
Author(s):  
S.E.J. Kaal ◽  
Winette T.A. Van Der Graaf ◽  
Carola J. C. Van Aart ◽  
Gerhard A. Zielhuis ◽  
Katja K. H. Aben

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 108-126 ◽  
Author(s):  
Maria Tsokos ◽  
Rita D. Alaggio ◽  
Louis P. Dehner ◽  
Paul S. Dickman

Ewing sarcoma/peripheral primitive neuroectodermal tumor (EWS/pPNET) and other tumors with EWS gene rearrangements encompass a malignant and intermediate neoplasm with a broad anatomic distribution and a wide age range but a predilection for soft tissue in children, adolescents, and young adults. The overlapping histologic, immunohistochemical and cytogenetic and molecular genetic features create diagnostic challenges despite significant clinical and prognostic differences. Ewing sarcoma is the 3rd most common sarcoma in children and adolescents, and desmoplastic small round cell tumor is a rare neoplasm that occurs more often in older children, adolescents, and young adults. Pathologic examination is complemented by immunohistochemistry, cytogenetics, and molecular genetics. This article reviews the clinicopathologic features of EWS/pPNET and desmoplastic small round cell tumor in the spectrum of tumors with EWS gene rearrangements. Other tumors with different histopathologic features and an EWS gene rearrangement are discussed elsewhere in this volume.


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