Stewart-Treves syndrome

Stewart-Treves syndrome refers to an angiosarcoma seen in the setting of lymphoedema 1.

It was classically attributed to lymphoedemas induced by radical mastectomy to treat breast cancer. Nowadays, we know that it can arise in any chronically lymphoedematous region due to any cause2

The incidence is unknown, but studies have shown that it occurred in a rate between 0.03% and 0.45% in patients who survive at least 5 years after radical mastectomy5,6.

Etiology

It can arise from any cause of chronic lymphoedema3,4:

  • mastectomy induced lymphoedema
  • Milroy disease
  • congenital lymphoedema
  • traumatic lymphoedema
  • filarial lymphoedema
  • elephantiasis nostras verrucosa
  • idiopathic lymphoedema
Physiopathology

Chronic lymphoedema seems to induce a degree of local immunodeficiency that leads to oncogenesis7.

Prognosis

The prognosis is very poor, because angiosarcomas are really agressive tumors that frequently recur locally and often metastasize early.

 

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Article information

rID: 2087
Section: Syndromes
Synonyms or Alternate Spellings:
  • Stewart Treves syndrome

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