Angiosarcoma arising from congenital primary lymphedema

Abstract

We herein report the case of a 3-year-old girl with atypical congenital right upper limb lymphedema who developed an angiosarcoma. Only a few cases have been reported following congenital form of lymphedema and only 4 in such a young child. We also summarize all cases of angiosarcoma associated with congenital lymphedema reported in the literature.

Keywords: Stewart-Treves syndrome; angiosarcoma; congenital; lymphangiosarcoma; lymphedema; sirolimus.