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Ewing sarcoma family of tumors in children younger than 10 years of age
Author(s) -
Huh Winston W.,
Daw Najat C.,
Herzog Cynthia E.,
Munsell Mark F.,
McAleer Mary Frances,
Lewis Valerae O.
Publication year - 2017
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26275
Subject(s) - medicine , sarcoma , cohort , radiation therapy , overall survival , distant metastasis , young adult , metastasis , pediatrics , surgery , cancer , pathology
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.