Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Childhood Malignant Extragonadal GCTs
Malignant GCTs located in the retroperitoneum or abdomen usually present in children before age 5 years; most tumors are of advanced stage and locally unresectable at diagnosis. A limited biopsy followed by platinum-based chemotherapy to shrink tumor bulk can lead to complete tumor resection in most patients. Despite advanced-stage disease in most patients, the 6-year EFS using PEB was 83% in the Pediatric Oncology Group/Children's Cancer Group intergroup study.
Though rare, benign and malignant GCTs can occur in the head and neck region, especially in infants. Often the airway is threatened. Surgery for nonmalignant tumors plus chemotherapy for malignant tumors can be curative.[Level of evidence: 3iiiDii]
Standard Treatment Options
Surgery: The role for surgery at diagnosis for extragonadal tumors is age- and site-dependent and must be individualized. Depending on the clinical setting, the appropriate surgical approach may range from no surgery (e.g., mediastinal primary tumor in a patient with a compromised airway and elevated tumor markers), to biopsy, to primary resection. In some cases, an appropriate strategy is biopsy at diagnosis followed by subsequent surgery in selected patients who have residual masses following chemotherapy.
Stages I and II
- Surgery and treatment with four to six cycles of standard PEB. These patients have an OS outcome greater than 90% with this regimen, suggesting that a reduction in therapy could be considered.[2,21]
- Surgery and treatment with six cycles of JEB.
Stages III and IV
- Surgery and treatment with four to six cycles of standard PEB. These patients have an OS outcome approaching 80% with this regimen.
- Surgery and treatment with six cycles of JEB yield similar OS.
Treatment Options Under Clinical Evaluation
The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.
- A completed COG trial investigated the addition of cyclophosphamide to standard-dose PEB. The results of this study are pending.
Current Clinical Trials
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood extragonadal germ cell tumor. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.
General information about clinical trials is also available from the NCI Web site.
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