Ewing sarcoma family of tumors in children younger than 10 years of age. Issue 4 (3rd October 2016)

• Record Type: Journal Article
• Authors: Huh, Winston W.; Daw, Najat C.; Herzog, Cynthia E.; Munsell, Mark F.; McAleer, Mary Frances; Lewis, Valerae O.
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 4(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2016-10-03
• Subjects: bone -- childhood cancer -- Ewing sarcoma -- pediatric -- sarcoma -- solid tumor; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26275
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Aim: Few data exist regarding the clinical characteristics and outcome of young children with Ewing sarcoma family of tumors (ESFT). Methods: We reviewed the records of ESFT patients at our institution younger than 10 years of age at diagnosis. Results: Forty‐two patients were identified. Median age was 6.4 years (range 0.6–9.5 years). Most patients had T2 (>5 cm) tumors (n = 31; 74%). Most common primary site was the extremity (n = 17; 41%). Seven patients (17%) had metastasis at diagnosis. For local tumor control, 20 patients had surgery only, 13 had radiation therapy only, and 6 had surgery plus radiation. Surgical margin status was negative in 19 patients (73%). Median follow‐up was 4.7 years (range 0.7–29.7 years), and 5‐year relapse‐free survival (RFS) and overall survival (OS) estimates were 67% (95% CI: 53–84%) and 82% (95% CI: 71–95%), respectively. Metastasis at presentation was the only significant predictor for decreased RFS ( P = 0.008) and OS ( P = 0.01). A trend was seen for T2 tumors with worse OS ( P = 0.09). Conclusion: Patients younger than 10 years of age with ESFT may have a better OS than older patients, but further study of a homogeneously treated larger cohort is needed.