Lymphangiosarcoma is an uncommon vascular tumor that usually develops in chronic lymphedema. The etiology of lymphangiosarcoma remains unknown. It develops mainly after mastectomy, in association with chronic lymphedema (Stewart-Treves syndrome), or after radiotherapy. The early diagnosis of this aggressive tumor is important, as it has a high risk of local recurrence and metastasis. Stewart-Treves syndrome occurs in 0.5% of patients who survive mastectomy for more than 5 years. The mean age at appearance of the angiosarcoma is 62 years, and the mean interval between mastectomy and the appearance of the tumour is 10.5 years. Only two cases have been reported in men following mastectomy. We present a 70-year old male patient with lymphangiosarcoma which developed three and a half years after the diagnosis of breast carcinoma. The patient underwent mastectomy of the right mammary gland with ipsilateral axillary lymph node dissection and had postoperative chemo- and radiotherapy.
Gonne E, Collignon J, Kurth W, et al. Angiosarcoma consecutive to chronic
lymphedema: a Steward-Treves syndrome. Rev Med Liege 2009;64:409-413.
Durr HR, Pellengahr C, Nerlich A, et al. StewartTreves syndrome as a rare complication of a hereditary lymphedema. Vasa 2004;33:42-45.
Lowenstein S. Der atiologische zusammenhang zwischen akutem ein-malignem trauma und sarkom. Z Klin Clir 1906;48:780-824.
Steward FM, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: A report of
six cases in elephantiasis chirurgica. Cancer 1948;1:64-81.
Oettle AG, van Blerk PJP. Postmastectomy lymphostatic endothelioma of Steward and Treves in a male. Br J Surg 1963;50:736-743.
Kirova YM, Gambotti L, De Rycke Y, et al.: A risk of second malignancies after adjuvant radiotherapy for breast cancer: a large-scale, single-institution review. Int J Radiat Oncol Biol Phys 2007;68(2):359-363.
Wierzibicka-Hainaut E, Guillet G. Steward-Treves syndrom (angiosarcoma on lymphedema): A rare complication of lymphedema. Press Med Dec 2010;39(12):1305-1308.
Herrman JB. Lymphangiosarcoma of the chronically edematous extremity. Surg Gynecol
Obstet 1965;121:1107-1115.
Schreiber H, Barry FM, Russell WC,et al. StewartTreves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surg 1979;114(1):82-85.
Sternby NH, Gynning I, Hogeman KE. Postmastectomy angiosarcoma. Acta Chir Scand
;121:420-432.
Styring E, Fernebro J, Jonsson PE, et al. Changing clinical presentation of angiosarcomas after breast cancer: from late tumors in edematous arms to earlier tumors on the thoracic wall. Breast Cancer Res Treat 2010;122(3):883-887.
McConnell AH, Haslam P. Angiosarcoma in postmastectomy lymphedema: report of five cases and review of the literature. Br J Surg 1959;46:322-332.
Caldwell JB, Ryan MT, Benson PM, et al. Cutaneous angiosarcoma arising in the radiation site of congenital hemangioma. J Am Acad Dermatol 1995;33:865-870
Mallon E, Powell S, Mortimer P, et al. Evidence for altered cell-mediated immunity in postmastectomy lymphedema. Br J Dermatol 1997;137:928-933.
Roy P, Clark MA, Thomas JM. Stewart-Treves syndrome-treatment and outcome in six
patients from a single center. Eur J Surg Oncol 2004;30:982-986.
Sharma A, Schwartz A. Stewart-Treves syndrome: Pathogenesis and management. J Am Acad Dermatol 2012;67:1342-1348.
Nascimento A, Raut C, Fletcher C. Primary angiosarcoma of the breast: clinicopathologic analysis of 49 cases, suggesting that grade is not prognostic. Am J Surg Pathol 2008;32:1896-1904.
Email this article (Login required) 
