HistoryThis section has been translated automatically.
Löwenstein, 1906; Stewart and Treves, 1948
DefinitionThis section has been translated automatically.
Originally classified as lymphangiosarcoma, this rare clinical variant of angiosarcoma manifests itself in areas of chronically persistent lymphedema of quite different genesis, and tends to metastasize early.
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Occurrence/EpidemiologyThis section has been translated automatically.
EtiopathogenesisThis section has been translated automatically.
- Whether (lymphatic) angiosarcomas originate from endothelial cells of the blood or lymph vessels is a controversial issue.
- Originally the syndrome was defined as lymphangiosarcoma after mastectomy for breast cancer! According to more recent approaches, it is considered a rare late complication of chronic lymphedema, independent of its genesis.
- Causes discussed include post-traumatic conditions, postoperative etiology (post-mastectomy syndrome), postoperative radiation, idiopathic, congenital or parasitic (e.g. in filariasis) etiology.
ManifestationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
Rough, initially light red, later deep red erythema that develops into hemorrhagic papules, plaques or nodules with a tendency to ulceration. Absence of or only slight pain. Prolonged persistence leads to confluent red-livid or blue-livid, coarse-elastic plaques or to disseminated seeding of the nodules over the affected extremity. In a later stage, tendency to extensive ulceration.
HistologyThis section has been translated automatically.
- Vascular spaces with atypical endothelial cells; inflammatory infiltrates, hemosiderin storage. Irregularly anastomosing, diffusely infiltrating vessels.
- Immunohistology: UEA 1, vimentin and HLA-DR positive.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
General therapyThis section has been translated automatically.
Radiation therapyThis section has been translated automatically.
Internal therapyThis section has been translated automatically.
Operative therapieThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Cui L et al (2015) Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature. Int J Clin Exp Pathol 8:11108-1115.
- Goetze S et al (2004) Successful therapy of Stewart-Treves syndrome with liposomal encapsulated doxorubicin. JDDG 2: 49-52
- Harrison WD et al (2015) Stewart-Treves syndrome following idiopathic leg lymphoedema: remember sarcoma. J Wound Care 24(6 Suppl):S5-7.
- Komorowski AL et al (2003) Angiosarcoma in a chronically lymphedematous leg: an unusual presentation of Stewart-Treves syndrome. South Med J 96: 807-808
- Löwenstein S (1906) The etiological connection between acute single trauma and sarcoma. Contribution by the surgeon 48: 780
- Mentzel T et al (2002) Tumors of the lymphatic vessel of the skin and soft tissue. Pathologist 23: 118-127
- Nakamura Y et al (2015) Long term control of pleural metastasis in Stewart-Treves syndrome with single-agent chemotherapies followed by maintenance chemotherapy. JDDG 13: 818-820
- Pitche P et al (2002) Stewart-Treves' syndrome: long term survival] Ann Dermatol Venereol 129: 236-237
- Stewart FW, Treves N (1948) Lymphangiosarcoma in postmastectomy lymphedema: a report of 6 cases in elephantiasis chirurgica. Cancer 1: 64-81
- Tse TS, Cooper LT (2001) Images in vascular medicine. Stewart's Treves angiosarcoma. Vasc Med 6: 267-268
Incoming links (9)Angiosarcoma in chronic lymphostasis; Cutaneous sarcomas (overview); Lymphangiokeratoma; Lymphangioma circumscriptum; Lymphangioma circumscriptum; Lymphangiosarcoma; Postmastectomy lymphangiosarcoma; Stewart-treves syndrome; Vimentin;
Outgoing links (11)Angiosarcoma (overview); Compression therapy; Cytostatics (overview); Doxorubicin; Gray; Kaposi's sarcoma (overview); Lymph drainage; Lymphedema (overview); Melphalan; Skin metastases; ... Show all
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