Stewart-Treves syndrome

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

It was classically attributed to lymphedemas induced by radical mastectomy to treat breast cancer. Nowadays, we know that it can arise in any chronically lymphedematous 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 lymphedema3,4:

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

Chronic lymphedema 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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