Stewart–Treves syndrome

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Stewart–Treves syndrome refers to a lymphangiosarcoma, a rare complication that forms as a result of chronic, long-standing lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer,[1] it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema.[2] Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment.[3] The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.[4]

History[edit]

It was discovered by Fred W. Stewart and Norman Treves in 1948.

Incidence[edit]

In the 1960s, the incidence 5 years after a radical mastectomy varied from 0.07% to 0.45%.[5] Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.[6]

Treatment[edit]

The treatment of choice is a large resection or amputation of the affected limb. Radiation therapy can precede or follow surgical treatment. Tumors that have advanced locally or have metastasized can be treated with mono or polychemotherapy, systemically or locally.[6] However, chemotherapy and radiation therapy have not been shown to improve survivorship significantly.[2]

Prognosis[edit]

Early detection is key. Untreated patients usually live 5 to 8 months after diagnosis.[2]

See also[edit]

References[edit]

  1. ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0. 
  2. ^ a b c Sharma, A; Schwartz, RA (June 2012). "Stewart-Treves syndrome: Pathogenesis and management.". J Am Acad Dermatol 67 (6): 1342–8. doi:10.1016/j.jaad.2012.04.028. PMID 22682884. 
  3. ^ Kumar MBBS MD FRCPath, Vinay (2010). Robins and Cotran: Pathologic Basis of Disease 8th Edition. Philadelphia, PA: Saunders Elsevier. p. 1093. ISBN 978-1-4160-3121-5. 
  4. ^ Pincus LB, Fox LP (August 2008). "Images in clinical medicine. The Stewart-Treves syndrome". N. Engl. J. Med. 359 (9): 950. doi:10.1056/NEJMicm071344. PMID 18753651. 
  5. ^ Heitmann, C; Ingianni, G (January 2000). "Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.". Ann Plast Surg. 44 (1): 72–5. doi:10.1097/00000637-200044010-00012. PMID 10651369. 
  6. ^ a b Wierzbicka-Hainaut, E; Guillet, G (December 2010). "[Stewart-Treves syndrome (angiosarcoma on lyphoedema): A rare complication of lymphoedema]". Presse Med. 39 (12): 1305–8. doi:10.1016/j.lpm.2010.06.017. PMID 20970956.