Lymphedema-associated Angiosarcoma

Chronic lymphedema of any origin is one of the most common risk factors for the development of angiosarcoma of skin and soft tissues. Lymphedema-associated angiosarcoma has been encountered in the following locations and clinical settings:

• Arms of elderly females who had modified radical mastectomy with axillary node dissection for breast cancer (Stewart-Treves Syndrome)
• Abdominal wall following lymph node dissection for carcinoma of penis
• Congenital, idiopathic, iatrogenic, traumatic, or filarial lymphedema affecting an extremity
• Localized massive lymphedema related to morbid obesity.

Clinical Features: The lesions develop on a background of non-pitting edema. Superficial lesions appear as red-purple plaques or multinodular growths that may coalesce and ulcerate in late stages. Lesions situated deep in the subcutis may only cause reddish-purple bruise-like mottling of the overlying skin. The areas of skin discoloration may extend distally to hands and feet or proximally to chest wall as the tumor dissects the tissue planes and spreads.