Lymphangiosarcoma is a rare malignant soft tissue neoplasm that originates from the lymphatic endothelial cells. It develops almost exclusively in tissues affected by chronic and longstanding lymphedema in various stages and with different etiologies. There are still differing opinions regarding the classification and characterization of this tumor, mainly because it shares significant histological similarities with other endothelial-origin neoplasms, such as angiosarcoma. In recent publications, considering these factors, it has been preferred to classify lymphangiosarcoma as angiosarcoma associated with chronic lymphedema (“angiosarcoma associated with lymphedema”). 

Lymphangiosarcoma:

Given the extreme invasiveness and malignancy of this neoplasm, the average survival time is around 18 months from diagnosis. Various therapies attempted thus far have not yielded satisfactory results. Currently, the only valid therapeutic solution appears to be the surgical “disarticulation” of the affected limb, with hopes of late metastasis of the neoplasm and early diagnosis. Encouraging, albeit preliminary, results have been reported recently with the technique of isolated limb hyperthermic perfusion using “melphalan.” It has demonstrated a significant reduction in the incidence of lymphangiosarcoma in patients who underwent rigorous medical-physical therapies and microsurgery for lymphedema. It seems that chronic and neglected stasis, with frequent lymphatic episodes, is the primary cause of malignant degeneration of the lymphatic endothelium. 

In conclusion, the aspect of prevention based on careful inspection of all lesions that arise on a lymphedematous limb, aiming for early diagnosis, combined with a long-term, combined treatment for lymphedema, currently represents the only means to combat this rare but formidable neoplasm. 

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