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    Childhood Extracranial Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Malignant Extragonadal Extracranial GCTs in Children

    Extragonadal extracranial germ cell tumors (GCTs) (i.e., sacrococcygeal, mediastinal, and retroperitoneal) are more common in children than in adults.[1] Children with extragonadal malignant GCTs, particularly those with advanced-stage disease, have the highest risk of treatment failure for any GCT presentation.[2,3]

    In a study of prognostic factors in pediatric extragonadal malignant GCTs, age older than 12 years was the most important prognostic factor. In a multivariate analysis, children aged 12 years or older with thoracic tumors had six times the risk of death compared with children younger than 12 years with primary nonthoracic tumors.[4]

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    Standard Treatment Options for Malignant Extragonadal Extracranial GCTs

    Standard treatment options for malignant extragonadal extracranial GCTs include the following:

    1. Surgery and chemotherapy.
    2. Biopsy followed by chemotherapy and possibly surgery.

    Outcome has improved remarkably since the advent of platinum-based chemotherapy and the use of a multidisciplinary treatment approach.[2,5] Complete resection before chemotherapy may be possible in some patients without major morbidity. For patients with locally advanced sacrococcygeal tumors, mediastinal tumors, or large pelvic tumors, tumor biopsy followed by preoperative chemotherapy can facilitate subsequent complete tumor resection and improve ultimate patient outcome.[5,6,7,8]

    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 be primary resection, biopsy before chemotherapy, or no surgery (e.g., mediastinal primary tumor in a patient with a compromised airway and elevated tumor markers). An appropriate strategy may be biopsy at diagnosis followed by chemotherapy and subsequent surgery in selected patients who have residual masses after chemotherapy.

    Stages I and II

    Surgery and chemotherapy with four to six cycles of standard cisplatin, etoposide, and bleomycin (PEB) is one treatment alternative. Patients treated with this regimen have an overall survival (OS) outcome greater than 90%, suggesting that a reduction in therapy could be considered.[2,9] An alternative treatment option is surgery and chemotherapy with six cycles of carboplatin, etoposide, and bleomycin (JEB).[5]

    Stages III and IV

    A treatment option for stage III and stage IV disease is surgery and chemotherapy with four to six cycles of standard PEB. These patients have an OS outcome approaching 80% with this regimen.[2] Another treatment option is surgery and chemotherapy with six cycles of JEB, which has a similar OS to the PEB regimen.[5]

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