Synonym(s)
angiosarcoma epithelioid; epithelioid angiosarcoma
DefinitionThis section has been translated automatically.
Rare, highly malignant, rapidly growing tumour originating from the blood vessels, which occurs mainly in the deep soft tissues and only rarely on the skin and internal organs (e.g. thyroid gland).
ManifestationThis section has been translated automatically.
Middle to advanced age; childhood is left out. Men are mostly affected.
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LocalizationThis section has been translated automatically.
Deep soft tissues; only rarely direct skin infestation.
Clinical featuresThis section has been translated automatically.
Rapidly growing, mostly solitary tumor of the deep soft tissues. Early hematogenic metastasis. Exophytic growth in the skin with formation of reddish-brown nodes.
HistologyThis section has been translated automatically.
- Diffuse laminar infiltration through large epithelioid cell clusters. Monomorphic epithelioid tumor cells with prominent vesicular nuclei and clearly protruding eosinophilic cytoplasm; recurrent intracytoplasmic vacuoles, occasionally with erythrocytes. Mature vascular lumina are rarely encountered.
- Immunohistology: Vascular markers (factor VIII; CD31) and cytokeratin positive.
- Cytogenetically, the detection of the translocation t(1;39(p36.3;q25) can be diagnostically decisive.
Differential diagnosisThis section has been translated automatically.
Clinical presentation: basal cell carcinoma, keratoacanthoma, squamous cell carcinoma Histological: epithelioid hemangiodothelioma.
TherapyThis section has been translated automatically.
Excision with large safety distance.
Progression/forecastThis section has been translated automatically.
Awkward. Life expectancy < 1 year. With purely dermal localization better prognosis!
LiteratureThis section has been translated automatically.
- Geller RL et al (2016) Cytologic features of angiosarcoma: A review of 26 cases diagnosed on FNA. Cancer Cytopathol doi: 10.1002/cncy.21726.
- Seo IS et al (2003) Postirradiation epithelioid angiosarcoma of the breast: a case report with immunohistochemical and electron microscopic study. Ultrastruct Pathol 27: 197-203
- Shi H et al (2015) Multifocal epithelioid angiosarcoma of the jejunum: a case report. Pathology 48:91-93.
- Sturgis EM et al (2003) Sarcomas of the head and neck region. Curr Opin Oncol 15: 239-252
- Tajima S et al (2015) ntimal sarcoma of the abdominal aorta and common iliac arteries presenting as epithelioid angiosarcoma of the skin: a case report. Int J Clin Exp Pathol 8:5856-5862.
- Wu J et al (2015) Epithelioid angiosarcoma: a clinicopathological study of 16 Chinese cases. Int J Clin Exp Pathol 8:3901- 3909.
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Incoming links (1)
Hemangioendothelioma epitheloides;Outgoing links (5)
Basal cell carcinoma (overview); Excision; Hemangioendothelioma epitheloides; Keratoakanthoma (overview); Squamous cell carcinoma of the skin;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
