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Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study
D Billmire1, C Vinocur, F Rescorla
1Section of Pediatric Surgery, J.W. Riley Hospital for Children, Indianapolis, Indiana 46202, USA.
Journal of Pediatric Surgery
|March 13, 2003
Summary
Pediatric malignant germ cell tumors in the abdomen and retroperitoneum show excellent survival rates with chemotherapy and surgery. Complete surgical removal after chemotherapy is crucial for optimal outcomes in these rare childhood cancers.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Medical Oncology
Background:
- Malignant germ cell tumors (GCTs) are rare in children.
- Abdominal and retroperitoneal GCTs represent a specific subset requiring tailored treatment strategies.
- Understanding optimal treatment sequencing is vital for improving outcomes.
Purpose of the Study:
- To evaluate survival and event-free survival (EFS) rates.
- To compare high-dose versus standard-dose cisplatin-based chemotherapy.
- To assess the role of surgical resection in conjunction with chemotherapy for abdominal/retroperitoneal GCTs.
Main Methods:
- A randomized study involving 25 pediatric patients with abdominal/retroperitoneal GCTs.
- Patients received etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB) chemotherapy.
- Surgical management included primary resection, delayed resection after chemotherapy, or biopsy.
Main Results:
- Overall 6-year EFS rate was 82.8% and survival rate was 87.6%.
- Survival rates were comparable between PEB (90.0%) and HDPEB (85.7%) groups.
- Most patients had advanced-stage disease (Stage IV in 17/25).
Conclusions:
- Pediatric abdominal/retroperitoneal GCTs have an excellent prognosis, even at advanced stages.
- Delayed surgical resection after chemotherapy is effective and important for differentiating viable tumor.
- Aggressive resection at diagnosis is not necessary; focus on complete post-chemotherapy removal.