In a 50-year-old patient with a malignant tumor of the left distal tibia (stage la, according to Enneking) undermining the cartilage of the ankle joint, an extremely rare primary leiomyosarcoma of bone was diagnosed. The diagnosis of a primary leiomyosarcoma of bone is possible only after a secondary (metastatic) leiomyosarcoma has been excluded. Furthermore, typical histopathological features, including immunohistochemical staining results, are required for the diagnosis. Surgery is the therapy of choice. An RO resection should always be attempted.
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