Sarcomas epithelioid hemangioendothelioma

Fetal fibroblasts myometrium vascular smooth muscle fetal myocardium vascular endothelial cells selected reticulum cells of lymphatic organs some CD30+ lymphoid cells (rarely) selected leiomyosarcomas, epithelioid hemangioendotheliomas, epithelioid angiosarcomas, primitive neuroectodermal tumors and Ewing s sarcomas, malignant peripheral nerve sheath tumors, and clear cell sarcomas... 

Wick MR, Manivel JG. Epithelioid sarcoma and epithelioid hemangioendothelioma An immunohistochemical and lectin-his-tochemical comparison. Virchows Arch A. 1987 410 309-316. 

Billings SD, Folpe AL, Weiss SW. Epithelioid sarcoma-like hemangioendothelioma. Am J Surg Pathol. 2003 27 48-57. 

FIGURE 13.37 Epithelioid sarcoma-like hemangioendothelioma of the subcutis. This tumor is unusual among endothelial lesions in that it is reproducibly keratin positive, but it lacks CD34. CD31 staining is also typical. 

This chapter will review rare primary malignant neoplasms such as hepatoblastoma, arising from hepatocytes cystoadenocarcinoma, arising from biliary cells angiosarcoma and other sarcomas as well as epithelioid hemangioendothelioma, arising from mesenchymal tissue and finally primary lymphoma, arising from lymphomatous tissue. 

Vascular tmnors Angiosarcoma Hemangiosarcoma Epithelioid hemangioendothelioma Leiomyosarcoma Fibrosarcoma Embryonal sarcoma Fibrous histiocytoma Lymphoma (Hodgkin s disease) Lymphoma (non-Hodgkin s disease)... 

It has accordingly been named epithelioid sarcomalike hemangioendothelioma (ESLH). Indeed, this entity shares immunoreactivity for keratin and vimentin with true epithelioid sarcoma (EPS), but ESEH paradoxically lacks CD34 (which is seen in EPS and most other hemangioendotheliomas) and additionally labels for CD31 and FLI-1. 

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