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.
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
Chronic lymphedema seems to induce a degree of local immunodeficiency that leads to oncogenesis7.
The prognosis is very poor, because angiosarcomas are really agressive tumors that frequently recur locally and often metastasize early.
- 1. Murphey MD, Fairbairn KJ, Parman LM et-al. From the archives of the AFIP. Musculoskeletal angiomatous lesions: radiologic-pathologic correlation. Radiographics. 1995;15 (4): 893-917. Radiographics (abstract) - Pubmed citation
- 2. Sharma A, Schwartz RA. Stewart-Treves syndrome: pathogenesis and management. (2012) Journal of the American Academy of Dermatology. 67 (6): 1342-8. doi:10.1016/j.jaad.2012.04.028 - Pubmed
- 3. Offori TW, Platt CC, Stephens M, Hopkinson GB. Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)--diagnostic beacons and a review of the literature. (1993) Clinical and experimental dermatology. 18 (2): 174-7. Pubmed
- 4. Hallel-Halevy D, Yerushalmi J, Grunwald MH, Avinoach I, Halevy S. Stewart-Treves syndrome in a patient with elephantiasis. (1999) Journal of the American Academy of Dermatology. 41 (2 Pt 2): 349-50. Pubmed
- 5. SCHIRGER A. Postoperative lymphedema: etiologic and diagnostic factors. (1962) The Medical clinics of North America. 46: 1045-50. Pubmed
- 6. Wierzbicka-Hainaut E, Guillet G. [Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of lymphoedema]. (2010) Presse medicale (Paris, France : 1983). 39 (12): 1305-8. doi:10.1016/j.lpm.2010.06.017 - Pubmed
- 7. Schreiber H, Barry FM, Russell WC, Macon WL, Ponsky JL, Pories WJ. Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency. (1979) Archives of surgery (Chicago, Ill. : 1960). 114 (1): 82-5. Pubmed