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    Extragonadal Germ Cell Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Nonseminoma

    Patients with nonseminomas should receive chemotherapy at diagnosis. These patients tend to have a very large tumor volume at diagnosis and are usually symptomatic. Initial debulking surgery is rarely useful. Many high-risk patients qualify for clinical trials. Standard therapy would generally be considered to be four courses of BEP (bleomycin, etoposide, and cisplatin).[1,2]

    A randomized study comparing four courses of BEP with four courses of VIP (etoposide, ifosfamide, and cisplatin) showed similar overall survival (OS) and time-to-treatment failure for the two regimens in patients with advanced disseminated germ cell tumors who had not received previous chemotherapy.[3,4][Level of evidence: 1iiA] Of the 304 patients on this study, 66 patients had extragonadal primary tumors, and in this subset of patients, responses were similar on the two regimens. Hematologic toxic effects in OS were substantially worse with the VIP regimen than with the BEP regimen.

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    Patients with a residual mass after chemotherapy may achieve long-term disease-free survival after postchemotherapy surgery with resection of all residual disease.[5][Level of evidence: 3iiiDii] Patients with nonseminomatous extragonadal germ cell tumors who relapse after front-line chemotherapy generally have poor prognoses with poor responses to salvage chemotherapy regimens, including autologous bone marrow transplantation, that have had success for recurrent testicular cancer.[6,7,8] Such patients, therefore, are candidates for studies of new approaches.

    Mediastinal Nonseminoma

    Mediastinal nonseminomas have certain unique aspects. The tumors are more frequent in individuals with Klinefelter syndrome and are associated with a risk of subsequent development of hematologic neoplasia that is not treatment related.[9,10] Approximately 50% of patients with mediastinal nonseminomas will survive with appropriate management.[11] High risk is partially related to tumor bulk, to chemotherapy resistance, and to a predisposition to develop hematologic neoplasia and other nongerm cell malignancies. In an uncontrolled study, some patients with a postchemotherapy residual mediastinal mass achieved long-term disease-free survival after complete resection, even when serum tumor markers were elevated.[5][Level of evidence: 3iiiDii] Patient selection factors may play a role in these favorable outcomes.

    Retroperitoneal Nonseminoma

    The prognosis of retroperitoneal nonseminoma is reasonably good and, similar to the situation with nodal metastasis from a testicular primary, is related to tumor volume.

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