Angiosarcoma in a limb with arteriovenous fistulas and elephantiasis.

Angiosarcoma is a rare malignant tumor of the soft tissues (less than 1% of sarcoma). It may affect various parenchymae (bones, liver, breast), the soft tissues or the skin. In the latter case it may be associated with lymphedema. Onset in the arm is typical with postmastectomy lymphedema (Stewart-Treves syndrome). The forms occurring in limbs with chronic lymphedema not associated with mastectomy are much rarer, with a total of 38 cases occurring in patients with an average age of 46.8 years and with lymphedema present for an average of 20 years. The survival rate for 5 years is 16.6%. The case observed by us had the characteristic onset of the malignant neoplasm on a pre-existing "angiomatosis lymphangiomatosis" pattern also featuring angiographycally detected arteriovenous fistula.