Stewart Treves Syndrome: literature review

Main Article Content

MD Lilem Valerio-Gil https://orcid.org/0000-0003-1005-3520
MD Vanessa Uclés-Villalobos https://orcid.org/0000-0002-8029-8163

Keywords

Stewart–Treves, angiosarcoma, syndrome

Abstract

Stewart-Treves syndrome is a very rare and lethal disease, characterized by infiltrative tumor lesions in limbs with lymphedema. More than 400 cases have been reported in the literature, with an incidence ranging from 0.07% to 0.45%. This syndrome mainly develops in patients with lymphedema secondary to radical mastectomy, radiotherapy, and axillary lymph node dissection. The pathophysiology is unclear, but local immunosuppression and angiogenesis are proposed mechanisms leading to malignant transformation. Its progression is aggressive, with local and metastatic spread. The differential diagnosis is supported by imaging studies and biopsy. The prognosis is poor, with a 5-year survival rate of 10% to 33%. Preventive therapy, including cellulitis treatment, proper lymphedema management, and compression use, is currently the most promising approach.

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