title: [Soft tissue sarcomas of bladder and prostate in children treated according to the CWS-96 protocol--report of the Polish Paediatric Solid Tumours Study Group].
abstract: OBJECTIVE
  analysis of the course, outcome and therapy complications in bladder/prostate soft tissue sarcomas (STS) in children treated from I'1997 to II'2003 according to CWS-96 protocol in Poland.
  
  
  METHODS
  22 children (M/F: 17/5, age: 8 months - 17 years 2 months; median 5,3y). Histopathology: RMS-20 patients (RME-14), non-RMS-2 patients.
  
  
  RESULTS
  96% presented with advanced neoplasm (III - 14, IV - 7). In 18 patients (82%) primary tumour exceeded 5cm; in 17 (77%) extended beyond site of origin. Lymph nodes metastases were stated in six (27%), distant metastases in seven patients (32%). No patient underwent primary complete tumour excision. All received chemotherapy for high risk group. Response to chemotherapy after three cycles was favourable in eight and unfavourable in eight children. Delayed resections were performed in 11 patients (mutilating - 7) proving complete in only five. Radiotherapy (32-50,4Gy) was given to 16 patients, mainly after delayed incomplete surgery. Five patients developed local relapse, four--continual disease progression. None of these nine patients had ever complete tumour resection. Nine patients died: seven due to neoplasm progression, two of therapy complications (septic shock, ARDS). Thirteen patients are alive (59%) with mean follow-up of 42 months. Eight children had bladder removed with continent ileal pouch formed in four.
  
  
  CONCLUSIONS
  1) Advanced stages of bladder/prostate sarcomas in children suggest the necessity of earlier diagnosis. 2) Due to adjuvant chemo- and radiotherapy approximately 60% of patients with this diagnosis may be nowadays cured of bladder/prostate sarcomas without mutilating surgical procedures.
authors.affiliation: Ośrodki Polskiej Pediatrycznej Grupy Guzów Litych: Klinika Pediatrii, Hematologii, Onkologii i Endokrynologii AM w Gdańsku. tsten@amg.gda.pl
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.forename: Ewa
authors.initials: E
authors.lastname: Bień
authors.forename: Bernarda
authors.initials: B
authors.lastname: Kazanowska
authors.forename: Anna
authors.initials: A
authors.lastname: Balcerska
authors.forename: Walentyna
authors.initials: W
authors.lastname: Balwierz
authors.forename: Alicja
authors.initials: A
authors.lastname: Chybicka
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Dłuzniewska
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Drozyńska
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Katski
authors.forename: Jerzy R
authors.initials: JR
authors.lastname: Kowalczyk
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kurylak
authors.forename: Jarosław
authors.initials: J
authors.lastname: Peregud-Pogorzelski
authors.forename: Dariusz
authors.initials: D
authors.lastname: Stencel
authors.forename: Beata
authors.initials: B
authors.lastname: Zalewska-Szewczyks
authors.forename: Jacek
authors.initials: J
authors.lastname: Wachowiak
authors.forename: Mariusz
authors.initials: M
authors.lastname: Wysockis
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D015653:Cystectomy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D011471:Prostatic Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D018714:Radiotherapy, Adjuvant; D012208:Rhabdomyosarcoma / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy; D016896:Treatment Outcome; D001749:Urinary Bladder Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000628:therapy