Stewart-Treves syndrome: Case report and literature review

Abstract

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

Keywords: Angiosarcoma; Breast cancer; Lymphangiosarcoma; Radiotherapy; Stewart-Treves syndrome.

Fig. 1

Large hematoma-like lesion on the inside of the right arm, centrally with erythema (a). One week later - highlighted solid infiltrate with a color change of the skin and tiny pink areas around (b).

Fig. 2

Varying growth patterns of the neoplastic proliferation could be recognized; irregular vessels dissecting the dermis, solid or infiltrative growth of neoplastic cells between collagen bundles were found (a). Immunostain for D2-40 (podoplanin) shows an intense staining of the neoplastic cells (b).

Fig. 3

FDG-PET/CT examination. 18F-FDG avid infiltration (arrow) of the skin and subcutaneous tissue at the right arm (A-axial CT image; B-axial fused image; C-coronal fused image; D-PET image).

Fig. 4

FDG-PET/CT examination. 18F-FDG avid infiltration (arrow) of the stump with involvement of the deltoid muscle, the latissimus dorsi muscle and the pectoralis muscle (A-axial CT image; B-axial fused image; C-axial PET image; D-coronal PET image).