Report of five cases and review of the literature. The lesion has a predilection for middle-aged men and for localization in the extremities. The mild and delayed nature of the symptoms contributes to delayed or inadequate treatment and an unusually high over-all recurrence rate of 50 per cent. En bloc resection is the treatment of choice. Amputation should be reserved for large tumors with extensive or intra-articular invasion, osseous erosion, or major neurovascular involvement.
Pulmonary lobectomy appears to be effective in treating localized pulmonary metastasis.
Chemotherapy and radiotherapy can also provide palliative benefits to patients with widespread metastases.