Murine Models Of Endothelial Cell Neoplasms

There are two murine models of endothelial cell neoplasms generated by subcutaneous injection of endothelial cells. One uses murine endothelial cells transformed with the middle T antigen of the murine polyoma virus, and the other uses endothelial cells derived from a spontaneously arising hemangioendothelioma (HE).22-26 The endothelial cells that are virally transformed are on a mixed MHC background (H-2d/H-2b), making them suitable for use only in SCID mice.26 The endothelial cells (EOMA) derived from the spontaneously arising HE are from the 129/J strain, which is commercially available (now called the 129P3/J) with a defined H-2b MHC background. EOMA cells have also been well characterized with regard to endothelial cell phenotype, protein expression, response to inhibitors of angiogenesis, and even with regard to the development of Kassabach—Merritt syndrome.19'20'22'27-31 The fact that the EOMA cell model is relatively well characterized and can be used with immunocompetent mice in a commercially available strain makes it the preferred model. The appearance of a 129P/3 mouse bearing an HE after EOMA injection is depicted in Figure 16.1.

FIGURE 16.1 Murine hemangioendothelioma. A 129P/3 mouse is shown 7 days after subcutaneous injection with EOMA cells. The large subcutaneous mass is a hemangioendothelioma (HE). The violaceous hue is due to blood trapped within the lesion. HE lesions form with 100% efficacy after subcutaneous injection with EOMA cells.

FIGURE 16.1 Murine hemangioendothelioma. A 129P/3 mouse is shown 7 days after subcutaneous injection with EOMA cells. The large subcutaneous mass is a hemangioendothelioma (HE). The violaceous hue is due to blood trapped within the lesion. HE lesions form with 100% efficacy after subcutaneous injection with EOMA cells.

FIGURE 16.2 Children with endothelial cell neoplasms. The first (left) case is that of a 10-month-old girl with an endothelial cell neoplasm involving the upper extremity. Bleeding and ulceration frequently accompany these lesions. Her lesion shows some signs of involution, as

evidenced by the gray, patulous skin in the central area of the lesion. Even with complete involution of the lesion, the site will never look like normal skin and will retain the gray color and texture difference seen in the central portion of this lesion. The other cases show facial lesions.

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