Title:
Stewart-Treves Syndrome Involving Chronic Lymphedema Postmastectomy: a Case Report and Review of the Literature.

Authors:
Vanesa Rodríguez-Fernández, MD; Lucía Cameselle-Cortizo, MD; Javier Valdés-Pons, MD, PhD; Alejandro Novo Domínguez, MD, PhD; Elena Figueiredo-Alonso, MD; Ángeles Corbillón Estévez, MD; Jorge F Cameselle-Teijeiro MD, PhD; Fernando Carlos Schmitt MD, PhD., Spain

Abstract:

Stewart-Treves syndrome (STS) is a rare and fatal entity, defined as the cutaneous angiosarcoma that arises in the context of long-standing chronic lymphedema. It usually occurs in women who develop lymphedema in the upper limb secondary to treatment for breast cancer (BC). It occurs in approximately 0.5% of patients who survive at least 5 years after radical mastectomy. Here, we present the case of an 84-year-old woman with chronic lymphedema secondary to BC surgery. She developed a purple macule over the area with chronic lymphedema 7 and a half years after BC diagnosis. Pathological results from biopsies of the lesion were consistent with angiosarcoma (STS). She received chemotherapy treatment followed by amputation of the affected limb. She died 18 months after diagnosis, due to local and distant spread of the disease. Despite the treatment, the prognosis remains severe with poor survival.