RESUMO
Presentation of an uncommon paratesticular tumour, located in the spermatic cord and originated in the smooth muscle of the cremaster, deferent, or the cord's vascular structures. In 1966, KYLE in Canada reviewed 22 cases of paratesticular leiomyosarcoma from the English literature, and in 1984 PUTS in the Netherlands extended this catalogue to 39. TAKAHA in 1991 presented 18 cases found in the Japanese literature. The theory of malignant degeneration from a previously existing leiomyomatous tumour is generally accepted. This entity is clinically associated with a clear tendency to local relapse, which does not imply a worse prognosis, and with a high percentage of blood metastasis three years after exeresis. The choice therapy is oncologic inguinal orchiectomy. Radiotherapy and chemotherapy, as coadjuvant therapies, appeared to be more effective in children's than in adult's sarcoma. Prognosis of these neoplasias in the reviewed literature is highly variable, ranging between 10% and 62% survival at 5 years, possibly due to the diversity of therapies and the patient's stage at the time of diagnosis.
