Paratesticular embryonal rhabdomyosarcoma was diagnosed in 4 children admitted to our hospital between 1973 and 1978. On the basis of these 4 cases and the reviewed literature the current concept in the management of this tumor is discussed. A combined multidisciplinary approach consisting of radical orchiectomy, retroperitoneal lymph node dissection and chemotherapy is necessary. When retroperitoneal lymph nodes are found to be involved by the tumor radiation therapy of this area should be admitted.

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