The treatment of choice is a large resection or amputation of the affected limb. Radiation therapy can precede or follow surgical treatment. Tumors that have advanced locally or have metastasized can be treated with mono or polychemotherapy, systemically or locally. However, chemotherapy and radiation therapy have not been shown to improve survivorship significantly. In cases of upper limbs, forequarter amputation (disarticulation of upperlimb along with clavicle and scapula) is preferred.
Stewart–Treves syndrome
Johnson–McMillin syndrome
Degos disease
Chandler's disease
Cruveilhier–Baumgarten disease
Nezelof syndrome
Van Den Bosch syndrome
Van Wyk and Grumbach syndrome