Angiosarcoma arising in a patient with Maffucci syndrome

Abstract

Maffucci syndrome comprises multiple enchondromas together with cutaneous haemangiomas. Review of the literature shows a 15-20% incidence of sarcomatous change in one or more of the enchondromas. Malignancy arising from the vascular component, however, has only been reported in one case. We present a further case where a patient with Maffucci syndrome presented with multifocal angiosarcoma.