[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].
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.
Ośrodki Polskiej Pediatrycznej Grupy Guzów Litych. ebien@amg.gda.pl
Bień
Ewa
E
Stachowicz-Stencel
Teresa
T
Kazanowska
Bernarda
B
Balcerska
Anna
A
Balwierz
Walentyna
W
Chybicka
Alicja
A
Dłuzniewska
Agnieszka
A
Drozyńska
Elzbieta
E
Kurylak
Andrzej
A
Matysiaks
Michał
M
Krawczuk-Rybak
Maryna
M
Rychłowska
Magdalena
M
Solarz
Elzbieta
E
Sopyło
Barbara
B
Stencels
Dariusz
D
Wachowiaks
Jacek
J
Wieczorek
Maria
M
Woźniak
Wojciech
W
Wysocki
Mariusz
M
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