PRIMARY EXTRAGONADAL GERM-CELL TUMORS - C LINICAL-FEATURES, DIFFERENTIAL-DIAGNOSIS AND TREATMENT

Background: Primary extragonadal germ cell tumors are a rare malignant disease in young males. They account for only 1 to 4% of all germ cel l tumors. Patients and Methods: In this paper we describe three select ed cases of primary extragonadal germ cell tumors. The literature is r eviewed with regard to clinical features, differential diagnosis and t reatment. Results: Tumor markers alpha-fetoprotein and human chorionic gonadotropin are of considerable diagnostic value if disease distribu tion is considered. With cisplatin-based combination chemotherapy simi lar disease-free survival rates are achieved as for testicular tumors with poor-prognosis metastatic disease. Surgical procedures play a rol e as adjunctive modality. Conclusions: If young males present with a m ass in the retroperitoneum or in the anterosuperior mediastinum, a pri mary extragonadal germ cell tumor should be taken into consideration. Tumors of both localisations have distinct clinical features but carry a similar prognosis. Patients benefit from the cumulative experience of a specialist unit.