Case Report

Angiosarcoma in a Chronically Lymphedematous Leg: An Unusual Presentation of Stewart-Treves Syndrome

Authors: Andrzej L. Komorowski, MD, Wojciech M. Wysocki, MD, Jerzy Mitus, MD, PHD

Abstract

Angiosarcoma arising from chronic lymphedema is referred to as Stewart-Treves syndrome. It typically occurs as a complication of long-lasting lymphedema of the arm after mastectomy and/or radiotherapy for breast cancer. Angiosarcoma associated with idiopathic lymphedema of the lower extremity is extremely rare. We report a case of diffuse angiosarcoma of the leg in a patient with a 25-year history of idiopathic lymphedema. Despite rapid aggressive surgical treatment, the patient died 6 weeks after diagnosis.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Enzinger FM, Weiss SW. Malignant vascular tumors, in Enzinger FM, Weiss SW (eds): Soft Tissue Tumors. St. Louis, C.V. Mosby, 1988, ed 2, pp 545–580.
 
2. Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema. Cancer 1948; 1: 64–81.
 
3. Caldwell JB, Ryan MT, Benson PM, James WD. Cutaneous angiosarcoma arising in the radiation site of congenital hemangioma. J Am Acad Dermatol 1995; 33: 865–870.
 
4. Nanus DM, Kelsen D, Clark DG. Radiation-induced angiosarcoma. Cancer 1987; 60: 777–779.
 
5. Azurdia RM, Guerin DM, Verbov JL. Chronic lymphedema and angiosarcoma. Clin Exp Dermatol 1999; 24: 270–272.
 
6. Hallel-Halevy D, Yerushalmi J, Grunwald MH, Avinoach I, Halevy S. Stewart-Treves syndrome in a patient with elephantiasis. J Am Acad Dermatol 1999; 41: 349–350.
 
7. Sinclair SA, Sviland L, Natarajan S. Angiosarcoma arising in a chronically lymphedematous leg. Br J Dermatol 1998; 138: 692–694.
 
8. Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma: A report of 67 patients and a review of the literature. Cancer 1996; 77: 2400–2406.
 
9. Fitzpatrick PJ. Lymphangiosarcoma in breast cancer. Can J Surg 1969; 12: 172.