Hemangiosarcoma of the left hand in a patient with the rare combination ofMaffucci's and Stewart Treves syndrome

We describe a patient with the previously unseen combination of Maffucci's and Stewart Treves syndrome who presented with art angiosarcoma of the hand Maffucci's syndrome is characterized by the presence of multiple enchondro ma and soft tissue hemangioma. The syndrome is a rare nonhereditary conditi on with a usual onset in childhood. Malignant transformations are a common feature of this syndrome. In 1948, Stewart and Treves first described six c ases of lymphangiosarcoma after radical mastectomy. This syndrome is an unu sual form of angiosarcoma occuring as a complication of lymphedema. Chronic lymphedema and lymphangiectasia preceding lymphangiosarcoma may not only b e induced by radical mastectomy with axillary lymph node dissection and pos toperative radiation therapy Posttraumatic, congenital or spontaneous chron ic lymphedema may also be associated with lymphangiosarcoma. A time interva l of many years seems to be required before malignant transformation develo ps. Generally the syndrome has a very poor prognosis. Both syndromes described above are of a rare frequency. We report this case because of prior unknown coincidence of both syndromes.