Epidemiology, clinical features and treatment outcome of Wilms' tumor in Taiwan: a report from Taiwan Pediatric Oncology Group.
OBJECTIVE
Taiwan Pediatric Oncology Group (TPOG)-W-91 is the first multi-institutional Wilms' tumor study for children in Taiwan. This clinical trial used a multidisciplinary approach, based on and similar to the National Wilms' Tumor Study 4. The study was conducted to evaluate the epidemiological characteristics and analyze the outcome of Wilms' tumor patients treated with this protocol.


METHODS
Ninety eight children with Wilms' tumor (WT) were analyzed for distributions of age, gender, associated congenital anomalies, tumor sites, histology, tumor weights, and clinical stages. Patients received individualized multimodality treatment based upon the histology of the tumor and clinicopathologic stage. The treatment included surgery, radiotherapy and 2-, 3-, and 4-agent active chemotherapeutic agents. Seventy patients were eligible for analysis of treatment outcome. The endpoints were progression-free and overall survival (PFS, OS). Patients were divided into various subgroups according to the chemotherapy regimen used, tumor stage, age at diagnosis, gender, and tumor weight. The prognostic factors were evaluated and the survival rates of various clinical subgroups were compared using log-rank test.


RESULTS
The average annual incidence rate of WT was 2.9 per million children under 15 years of age. The M/F ratio was 1.04. The mean age at diagnosis was 3.7 years. All bilateral tumors occurred in females. Congenital anomalies were present in 17.3% of patients. Anaplastic histology was found in 6 of 98 cases (6.1%). The stage distribution was: I, 43.2%; II, 19.3%; III, 23.9%; IV, 6.8%; and V, 6.8%. The median follow-up time was 89.1 months (range, 1.8 to 128.1 months). The 5-year PFS rate was 0.7841 (SE, 0.0494; 53 of 70 patients) and the 5-year OS rate was 0.886 (SE, 0.038; 63 of 70 patients). Gender was found to be the only significant prognostic variable.


CONCLUSIONS
This study evaluated the epidemiological characteristics, clinical features, multimodality therapy regimens, and treatment outcome of WT in Taiwan. Data obtained from this study may lead to further improvement in the prognosis of pediatric malignant solid tumor.
Hung
Iou-Jih
IJ
Division of Hematology/Oncology, Chang Gung Children's Hospital, Linkou, Taiwan.
Chang
Wan-Hui
WH
Yang
Chao-Ping
CP
Jaing
Tang-Her
TH
Liang
Der-Cherng
DC
Lin
Kai-Hsin
KH
Lin
Dong-Tsamn
DT
Hsiao
Chih-Cheng
CC
Hsieh
Yuh-Lin
YL
Chen
Jiann-Shiuh
JS
Chang
Tai-Tsung
TT
Peng
Ching-Tien
CT
Shu
San-Ging
SG
Lin
Ming-Tsan
MT
Chen
Bow-Wen
BW
Lin
Kuo-Sin
KS
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007680:Kidney Neoplasms / Q000453:epidemiology / Q000628:therapy*; D008297:Male; D016016:Proportional Hazards Models; D016019:Survival Analysis; D013624:Taiwan / Q000453:epidemiology; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000453:epidemiology / Q000628:therapy*