In bone, ESFT arise with equal frequency in the appendicular and the axial skeletons, commonly involve the diaphysis, and are more common in flat bones than is osteosarcoma [51]. The femur is the most common long bone involved, followed by the tibia, fibula, and humerus. Bones of the pelvis are the next most common group involved. Nevertheless, ESFT may affect any bone, including the ribs, scapula, spine, clavicle and skull [5,6,52]. Extraosseous ESFT may arise anywhere in the body, including soft tissue, skin, and visceral organs [