is a rare malignant tumor which occurs in long-standing cases of Primary or Secondary Lymphedema
. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.
The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical Halstedian radical mastectomy, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960's to early 1970's, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment - and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as Stewart-Treves syndrome
(which can be both lymphangiosarcoma and hemangiosarcoma
- Cancer: Principles & Practice of Oncology, 6th Edition, DeVita, Hellman and Rosenberg, p. 1853