Ewing sarcoma family of tumors in children younger than 10 years of age

• Published in: Pediatric blood & cancer 2017-04, Vol.64 (4), p.np-n/a
• Main Authors: Huh, Winston W., Daw, Najat C., Herzog, Cynthia E., Munsell, Mark F., McAleer, Mary Frances, Lewis, Valerae O.
• Format: Article
• Language: English
• Subjects: Age; bone; Bone Neoplasms - secondary; Bone Neoplasms - therapy; Child; Child, Preschool; childhood cancer; Combined Modality Therapy; Ewing sarcoma; Female; Follow-Up Studies; Hematology; Humans; Infant; Infant, Newborn; Lymphatic Metastasis; Male; Metastasis; Neoplasm Staging; Oncology; pediatric; Pediatrics; Prognosis; Retrospective Studies; sarcoma; Sarcoma, Ewing - pathology; Sarcoma, Ewing - therapy; solid tumor; Survival Rate; Tumors
• ISSN: 1545-5009; 1545-5017
• DOI: 10.1002/pbc.26275

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.