Malignant fibrous histiocytoma occurs rarely in the genitourinary organs, and is exceptional in the spermatic cord. The prognosis of deep malignant fibrous histiocytoma not occurring in subcutaneous tissue or fascia is generally poor. We report a case, of malignant fibrous histiocytoma giant cell type, arising around the spermatic cord. The patient is alive without metastasis ten years after initial therapy. To our knowledge, this is the longest reported survivor of this disease. Radical inguinal orchiectomy along with wide en bloc local resection is the therapy of choice.
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