Citation, DOI, disclosures and article data
Citation:
D'Souza D, Bickle I, Qureshi P, et al. Stewart-Treves syndrome. Reference article, Radiopaedia.org (Accessed on 16 Apr 2024) https://doi.org/10.53347/rID-2087
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 chronically lymphedematous regions of any cause 2.
The incidence is unknown, but studies have shown that it occurred at a rate of between 0.03% and 0.45% in patients who survive at least 5 years after radical mastectomy 5,6.
Pathology
Etiology
It can arise from any cause of chronic lymphedema 3,4:
Pathophysiology
Chronic lymphedema seems to induce a degree of local immunodeficiency that leads to oncogenesis 7.
Treatment and prognosis
The prognosis is very poor because angiosarcomas are very aggressive tumors that frequently recur locally and often metastasize early.
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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
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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
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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
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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
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5. Scherger A. Postoperative lymphedema: etiologic and diagnostic factors. (1962) The Medical clinics of North America. 46: 1045-50. Pubmed
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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
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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
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