Lymphangiosarcoma is a rare malignant soft tissue tumor originating from lymphatic endothelium cells. It grows almost exclusively in tissues affected by chronic incurable lymphedema of various stages and different etiology. The classification and staging of this tumor is still much debated, especially because, from a pathological anatomy point of view, it has many similarities with other tumors of endothelial origin, such as, for example, with hemangiosarcoma. Therefore, considering these elements, in more recent publications, lymphangiosarcoma has been classified as angiosarcoma associated with lymphedema. Because of its aggressive and malignant nature, the average survival is around 18 months after diagnosis. No therapy attempted so far has led to satisfactory results; for now, the only valid therapeutical solution is still surgical “disarticulation” of the affected limb, while hoping that, through early diagnosis, the tumor has not yet metastisized. Some encouraging, although still preliminary results, have recently been reported with hyperthermic perfusion of the isolated extremity with “melphalan”. A significant reduction has been reported in the incidence of lymphangiosarcoma in patients undergoing rigorous medical-physical therapies and microsurgery to treat lymphedema. Indeed, inveterated and neglected lymph stasis, with recurrent and frequent lymphangitic episodes, is allegedly the main agent responsible for cancerous degeneration of the lymphatic endothelium.

Therefore, in conclusion, prevention based on accurate inspection of all lesions developed on a lymphedematous limb, and aimed at early diagnosis, as well as combined, prolonged treatment of lymphedema, are still the only weapons to fight this rare yet intimidating cancer.