Primary Leiomyosarcoma - Bone

Primary leiomyosarcoma of bone is a rare entity. It is thought to originate from vascular smooth muscle cells or the mesenchymal fibroblasts within the bone. Metastases from uterus and gastrointestinal tract must be excluded. When arising in long bones, amputation is the mainstay of therapy. Majority of patients die of pulmonary metastases.