[Paratesticular rhabdomyosarcoma in children. Is retroperitoneal lymphadenectomy necessary in disease limited to the scrotum?].

Between 1970 and 1993, five children with paratesticular rhabdomyosarcoma (PR) and age ranging from 3 to 10 years were treated by our group. Following radical orchidectomy and clinical staging, 4 were rates as Group I-IRS (completely resected local disease) and 1 in Group IV-IRS (distant metastasis). All patients in Group I underwent intensive chemotherapy with vincristine, actynomicin D and cyclophosphamide (VAC) for 18 months, while no case of retroperitoneal lymphadenectomy was performed. Currently, all patients are disease free with a mean follow-up of 8 years and 5 months. The single patient included in Group IV received radiotherapy (800 rads in abdominal field) and chemotherapy with VAC + prednisone (PDR), and retroperitoneal lymphadenectomy of residual masses was performed. The patient developed liver metastasis and died 20 months later. The purpose of this paper is to convey the reader our experience in the treatment of PR in children, making a special reference to the excellent prognosis when the disease is limited to the scrotum and recommending in such cases the use of intensive chemotherapy with VAC after performing radical orchidectomy.