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[Genitourinary soft tissue sarcomas located outside bladder and prostate in children treated according to the CWS-96
1, Ewa Bień, Teresa Stachowicz-Stencel
1Ośrodki Polskiej Pediatrycznej Grupy Guzów Litych. ebien@amg.gda.pl
Insights
Pediatric genitourinary sarcomas, excluding bladder and prostate, show a favorable prognosis with current therapies. Chemotherapy and radiotherapy are effective, though severe myelosuppression may occur.
Area of Science:
- Pediatric Oncology
- Genitourinary Oncology
- Sarcoma Research
Background:
- Genitourinary sarcomas in children are rare but aggressive tumors.
- Treatment protocols have evolved to improve outcomes.
Purpose of the Study:
- To analyze the efficacy of the CWS-96 protocol in treating non-bladder/prostate genitourinary sarcomas in children.
- To evaluate treatment outcomes, including survival rates and complications.
Main Methods:
- Retrospective analysis of 19 children treated between 1997 and 2003.
- Histopathological diagnosis included rhabdomyosarcoma (RMS) and non-RMS subtypes.
- Treatment involved surgery, chemotherapy, and radiotherapy.
Main Results:
- Rhabdomyosarcoma was the most common type (15/19 patients).
- Testes and paratesticular regions were the most frequent primary sites.
- Despite incomplete primary excision in 12 patients, 84% survived with a median follow-up of 48 months.
- Chemotherapy showed efficacy in patients with residual tumor after surgery.
Conclusions:
- Favorable prognosis for pediatric genitourinary sarcomas, even with incomplete initial surgical resection.
- Chemotherapy and radiotherapy were associated with severe but transient myelosuppression.
- Mutilating surgery was the primary cause of permanent complications.
Aim:
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.
Material And 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.
