Hemangiosarcoma of the left hand in a patient with the rare combination of Maffucci's and Stewart Treves syndrome.
We describe a patient with the previously unseen combination of Maffucci's and Stewart Treves syndrome who presented with an angiosarcoma of the hand. Maffucci's syndrome is characterized by the presence of multiple enchondroma and soft tissue hemangioma. The syndrome is a rare nonhereditary condition with a usual onset in childhood. Malignant transformations are a common feature of this syndrome. In 1948, Stewart and Treves first described six cases of lymphangiosarcoma after radical mastectomy. This syndrome is an unusual form of angiosarcoma occuring as a complication of lymphedema. Chronic lymphedema and lymphangiectasia preceding lymphangiosarcoma may not only be induced by radical mastectomy with axillary lymph node dissection and postoperative radiation therapy. Posttraumatic, congenital or spontaneous chronic lymphedema may also be associated with lymphangiosarcoma. A time interval of many years seems to be required before malignant transformation develops. 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.
Bach
A D
AD
Department of Plastic and Hand Surgery, University of Freiburg, Germany.
Walgenbach
K J
KJ
Horch
R E
RE
D000671:Amputation, Surgical; D003952:Diagnostic Imaging; D004687:Enchondromatosis / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D005260:Female; D006225:Hand / Q000473:pathology; D006394:Hemangiosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008204:Lymphangiosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D008209:Lymphedema / Q000175:diagnosis; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery