Stewart-Treves Syndrome

  • Stewart-Treves syndrome:
    • Is a rare complication of longstanding lymphedema.
  • Clinical suspicion should be high:
    • As this syndrome is easily misdiagnosed and treatment is advertently delayed
  • Once diagnosed:
    • Surgical excision is the treatment of choice:
      • Sometimes requiring forequarter amputation
  • Chemotherapy may have some role:
    • However its benefit is unclear.
  • Multiple studies have shown the 5-year survival to be very poor:
    • At less than 10%.
    • The mean survival is 20 months.
  • References
    • Cui L, Zhang J, Zhang X, et al. Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. Int J Clin Exp Pathol. 2015;8(9):11108-11115.
    • Penel N, Bui BN, Bay JO, et al. Phase II trial of weekly paclitaxel for unresectable angiosarcoma: the ANGIOTAX Study. J Clin Oncol. 2008;26(32):5269-5274.

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