Lymphangiosarcoma in chronic hereditary oedema (Milroy's disease)

Lymphangiosarcoma arising in chronic lymphoedema is extremely rare. In a reference population of about four million people, during a thirty year period (1957-1987), only four patients were treated for such a tumour. The neoplasm is almost exclusively seen in elderly patients after mastectomy but in two of our patients, reported in this paper, it arose in chronic hereditary oedema (Milroy's disease). In both these patients there was a considerable treatment delay because of wrong diagnosis. The tumour extent was difficult to assess macroscopically and ablative surgery had to be a disarticulation of the involved extremity. Macular or papular purple lesions in a lymphoedematous extremity should be a manifestation of this aggressive neoplasm.