A paratesticular pleomorphic rhabdomyosarcoma occurring in a 63-year old man is reported. Clinical signs were like any intrascrotal tumor. CT-scan and histological examination with immunohistochemical study were necessary for the diagnosis and stadification of this cancer. The patient underwent a radical inguinal orchidectomy. The patient developed a local recurrence, which was treated by radiotherapy, and subsequently developed metastases two years later, which were treated by chemotherapy.
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