Long-term survival results of pediatric rhabdomyosarcoma patients: a single-center experience from Turkey.
The characteristics and clinical outcomes of 409 children with rhabdomyosarcoma, treated in a single center between 1972 and 2003, were evaluated to identify those characteristics that may have improved the outcome. Retrospective analysis was performed on 409 children for variables such as age, sex, primary tumor site, TNM (Tumor, Node, Metastasis) stage, Intergroup Rhabdomyosarcoma Studies (IRS) clinical group, histological subtypes, treatment. The mean age was 5.4 years and the male/female ratio was 1.6/1.0. Most of the patients were at stage III and the most common site of metastases was the lung. The median time of follow-up was 114 months. The 10-year event-free survival and overall survival rates were 27% and 33%, respectively. All parameters apart from sex and histological subtypes were found to be correlated with clinical outcome. Orbital and genitourinary system tumors (bladder-prostate tumors excluded), patients >1 year of age, TNM stage I and II tumors, IRS clinical group I and II tumors, grossly resectable tumors, and treatment with chemotherapy protocols containing anthracyclines correlated with better prognoses. In multivariate analyses, tumor invasion to surrounding tissue, regional lymph node involvement, and debulking surgery were found to be negatively correlated with prognosis. In children with rhabdomyosarcoma, survival rates can be improved owing to factors such as advancement in diagnostic and therapeutic techniques, improved supportive care, and a multidisciplinary approach.
Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey.
Canan
C
Akyüz
Neriman
N
Sari
Bilgehan
B
Yalçin
Ali
A
Varan
Tezer
T
Kutluk
Münevver
M
Büyükpamukçu
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000188:drug therapy* / Q000401:mortality*; D015996:Survival Rate; D014421:Turkey / Q000453:epidemiology