[Clinical report on diagnosis and treatment of 14 cases with pediatric Ewing's sarcoma family tumors].
OBJECTIVE
To recognize and improve the outcome of childhood Ewing's sarcoma family tumors, and to identify the efficacy and safety of the chemotherapy using RS-2002 Protocol.


METHODS
From September 1997 to September 2006, 14 newly diagnosed patients with the tumors were admitted, 9 were boys, and 5 were girls, the median age was 7.04 years, ranging from 1.58 years to 11.67 years. Among them, 9 patients were younger than 10 years. By the time of diagnosis, 9 patients had local diseases, and the other 5 patients had metastatic diseases. All the patients' diagnoses were confirmed by pathological studies. Nine patients had Ewing's sarcoma by histology, and the other 5 patients had peripheral primitive neuroectodermal tumors (PPNET). All of the patients were treated with multidisciplinary therapy, and RS-2002 Protocol for chemotherapy was used to treat patients with rhabdomyosarcoma in our hospital. Surgery and irradiation were performed for local control. Imaging studies were used for evaluation, reevaluation and follow-up.


RESULTS
Till April 30th 2007, 13/14 patients survived. The median follow-up time was 41 months (range: 7 months-115 months). The 10-year overall survival (OS) was 88.9%+/-10.5%, and the 10-year disease-free-survival (DFS) was 72.2%+/-13.8%; 3/14 patients had disease relapse, the median time to relapse from initial diagnosis was 23 months (range: 16-30 months). One patient developed second malignancy. No therapy related death was documented.


CONCLUSIONS
Childhood Ewing's sarcoma family tumors were not very rare, and the prognosis was acceptable with optimal treatment. RS-2002 Protocol was effective and safe in treating such patients.
Pan
Ci
C
Department of Hematology/Oncology, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200127, China.
Tang
Jing-yan
JY
Xue
Hui-liang
HL
Chen
Jing
J
Dong
Lu
L
Zhou
Min
M
Ye
Qi-dong
QD
Wang
Yao-ping
YP
D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012512:Sarcoma, Ewing / Q000628:therapy*; D016896:Treatment Outcome