ANGIOSARCOMA IN CONGENITAL HEREDITARY LYMPHEDEMA (MILROYS DISEASE) - DIAGNOSTIC BEACONS AND A REVIEW OF THE LITERATURE

In cases of congenital lymphoedema the finding of ulceration, violaceous nodules or papules, or apparent traumatic ecchymoses1 should act as a diagnostic beacon warning of dangers. A case is reported of a high-grade angiosarcoma developing in a patient with congenital hereditary lymphoedema (Milrov's disease). This is the second paper to report this complication, the third case report and the first case in which the diagnosis is substantiated by immunohistochemistry and lectin histochemistry. A review of cases of angiosarcoma complicating congenital hereditary and non-hereditary lymphoedema is also presented.