Abstract
tLymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prog-nosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, includingaxillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account forapproximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema.
Lym-phatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developingSTS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema.
Chroniclymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillarynodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction inthe incidence of chronic lymphedema from 40% to 4%.
The use of sentinel lymph node biopsy techniqueis also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STSdecreased significantly with improved surgical and radiation techniques.
The overall prognosis of STSpatients is very poor. Only early radical surgical removal, including amputation or disarticulation of theaffected limb, or wide excision at an early stage offers the greatest chance of long-term survival.
Only afew case reports and series with a small number of patients with lymphangiosarcoma can be found in theliterature. We present a case report of the first diagnosed STS at our department in an effort to highlightthe need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.