Síndrome de Stewart-Treves: linfangiosarcoma en linfedema crónico posmastectomía

Summary Introduction Lymphangiosarcoma is an angiosarcoma that develops on areas where there is chronic lymphedema, and is nearly always post-mastectomy (Stewart-Treves syndrome). It shows an incidence of 0.45% among patients that survive more than five years after the intervention. It is characterised by the slow development of hard reddish or purplish nodules, with a tendency towards spontaneous bleeding, that grow and multiply quickly and produce metastasis in the lungs. The therapeutic possibilities (radiotherapy, chemotherapy, amputation) are very aggressive and offer poor results, with a survival rate of less than 5% from the moment of their diagnosis. Case report A 71-year-old patient who was submitted to a mastectomy on the right-handside in 1989with axillary dissection, chemotherapy and radiotherapy of the lymph nodes in the supraclavicular and axillary regions, as well as in the wall of the thoracic cavity. She slowly developed a lymphedema in the same limb. Ten years after the intervention, some fast-growing purplish lesions appeared on the arm, and became nodular and had a tendency to bleed. After the biopsy examination, they were diagnosed as lymphangiosarcoma. The study for metastatic extension was negative. After assessing the patient's situation in the Oncology Unit, it was decided that the most resolvent therapy was the amputation of the limb. The patient refusedsuch treatment andreceivedpalliative attention until her death six months after diagnosis. Conclusions In spite oftheir aggressiveness, the therapies that were implemented-radiotherapy, chemotherapy and amputation of the limb- did not improve survival.