title: [Genitourinary soft tissue sarcomas located outside bladder and prostate in children treated according to the CWS-96 protocol--report from the Polish Paediatric Solid Tumours Study Group].
abstract: OBJECTIVE
  Analysis of therapy efficacy in non-bladder/prostate genitourinary sarcomas in children treated from I'1997 to VI'2003 with CWS-96 protocol in Poland.
  
  
  METHODS
  19 children (M/F: 15/4, age: 3m-17,5y; median 7,2y). Histopathology: RMS - 15pts (RME-13), non-RMS- 4.
  
  
  RESULTS
  Primary site: testes - 9 patients, paratesticular region - 6, uterus - 2, vagina and ovary-1 of each. 63% presented with low stage neoplasm (I - 7, II - 5). Primary tumour exceeded 5cm and/or invaded surrounding tissues in 7 patients (37%). 3 patients had regional, 2 patients--distant lymph nodes metastases. Primary excision: complete in 7 patients, incomplete - 12 (microscopically - 5, macroscopically - 7). Six of 7 patients with macroscopic tumour residues responded to chemotherapy (CR-4, GR-2). One patient (stage III triton tumour of uterus) did not, respond but obtained complete remission after mutilating delayed surgery. No other patient required delayed tumour resection. Radiotherapy (23,5-54 Gy) was given to 8 patients. 3 children developed local relapse, 3 patients died (16%): 2 due to neoplasm progression, 1 of neutropenia-related sepsis. 16 patients are alive (84%) with mean follow-up 48 months. The only permanent complications result from mutilating surgery.
  
  
  CONCLUSIONS
  1) prognosis in children with non-bladder/prostate genitourinary sarcomas is favourable despite incomplete primary excision of the neoplasm. 2) chemotherapy and radiotherapy were accompanied by severe but transient myelosupression in the HR group.
authors.affiliation: Ośrodki Polskiej Pediatrycznej Grupy Guzów Litych. ebien@amg.gda.pl
authors.forename: Ewa
authors.initials: E
authors.lastname: Bień
authors.forename: Teresa
authors.initials: T
authors.lastname: Stachowicz-Stencel
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: Andrzej
authors.initials: A
authors.lastname: Kurylak
authors.forename: Michał
authors.initials: M
authors.lastname: Matysiaks
authors.forename: Maryna
authors.initials: M
authors.lastname: Krawczuk-Rybak
authors.forename: Magdalena
authors.initials: M
authors.lastname: Rychłowska
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Solarz
authors.forename: Barbara
authors.initials: B
authors.lastname: Sopyło
authors.forename: Dariusz
authors.initials: D
authors.lastname: Stencels
authors.forename: Jacek
authors.initials: J
authors.lastname: Wachowiaks
authors.forename: Maria
authors.initials: M
authors.lastname: Wieczorek
authors.forename: Wojciech
authors.initials: W
authors.lastname: Woźniak
authors.forename: Mariusz
authors.initials: M
authors.lastname: Wysocki
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008207:Lymphatic Metastasis; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012208:Rhabdomyosarcoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000556:secondary / Q000601:surgery; D012509:Sarcoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000601:surgery; D016896:Treatment Outcome; D014565:Urogenital Neoplasms / Q000175:diagnosis* / Q000188:drug therapy* / Q000601:surgery