[Results of the treatment of paediatric non-rhabdomyosarcoma. One centre experience].
Non-rhabdomyosarcoma soft tissue sarcomas (NR STS) are a rare group of neoplasms of mesenchymal origin. The incidence of these tumours in children is low and due to it's heterogeneity and different response to chemotherapy and radiotherapy, unified treatment methods have not yet been established. THE AIM of our study was to analyze methods and treatment results of patients with NR STS treated in our centre.


METHODS
Between 1996 and 2004, 64 patients with NR-STS, aged 2.5-21.5 yrs, were treated in our institution. Treatment protocol included primary tumour resection or biopsy, induction (neoadjuvant) chemotherapy, local treatment: surgery and/or radiotherapy and adjuvant chemotherapy. Results of treatment were analyzed in relation to stage, tumour diameter, extent of surgery and response to chemotherapy.


RESULTS
Out of 64 patients, 48 are alive (75%), with a median observation time 4 yrs 3 m. Sixteen patients died: 1 of treatment complications, the rest from basic disease. Four years overall (OS) and event free survival (EFS) are 75% and 64% respectively. Early stage, tumour size less than 5 cm in diameter, radical surgery, complete and very good response to induction chemotherapy had a significant influence on survival.


CONCLUSIONS
Our results indicate that besides stage and tumour size, radical surgery played key role in the treatment of NRMSSTS and that radical resections were possible to perform after induction chemotherapy in 33% of patients with primarily unresectable tumours. High number of patients with stage IV disease at diagnosis, occurrence of distant relapses and good response to chemotherapy indicate the necessity for the use of chemotherapy in patients with NR STS.
Wakulińska
Anna
A
Klinika Onkologii, Instytut Pomnik Centrum Zdrowia Dziecka, Al. Dzieci Polskich 20, 04-730 Warszawa, Poland.
Dembowska-Bagińska
Bozenna
B
Cienciała
Małgorzata
M
Bothur-Nowacka
Joanna
J
Kościesza
Andrzej
A
Pakuła-Kościesza
Iwona
I
Perek
Danuta
D
D000293:Adolescent; D000328:Adult; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D011044:Poland / Q000453:epidemiology; D018714:Radiotherapy, Adjuvant; D012509:Sarcoma / Q000188:drug therapy / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome