Presentation of the case of one patient with a leiomyosarcoma of the spermatic cord with presence of numerous giant cells, a remarkably unusual finding. Presence of this type of cells, as well as their positivity for histiocytic markers in immunohistochemical staining, raise considerable problems for the differential diagnosis of this neoplasia with malignant fibrous histiocytomas, which probably requires a different, more aggressive therapeutical approach.

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