[Primary extragonadal germ cell tumors. Clinical manifestations, differential diagnosis and therapy].

BACKGROUND

Primary extragonadal germ cell tumors are a rare malignant disease in young males. They account for only 1 to 4% of all germ cell tumors.

PATIENTS AND METHODS

In this paper we describe three selected cases of primary extragonadal germ cell tumors. The literature is reviewed with regard to clinical features, differential diagnosis and treatment.

RESULTS

Tumor markers alpha-fetoprotein and human chorionic gonadotropin are of considerable diagnostic value if disease distribution is considered. With cisplatin-based combination chemotherapy similar disease-free survival rates are achieved as for testicular tumors with poor-prognosis metastatic disease. Surgical procedures play a role as adjunctive modality.

CONCLUSIONS

If young males present with a mass in the retroperitoneum or in the anterosuperior mediastinum, a primary 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.