Case report 569: Lymphangiosarcoma of the right upper extremity.
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A Sarcoma arising in the abdominal cavity in an aged Fischer-344 rat was studied by immunohistochemistry and electron microscopy. The white-yellow soft mass was located on the lumbosacral vertebrae, compressing adjacent parenchymal organs. The tumor was made up of spindle shaped cells situated in a background of myxoid substance and a small amount of reticulin and collagen fibers. The tumor cells grew in a loose storiform pattern and often adhered to each other by their cell processes to form ovoid or slitlike spaces. Immunohistochemically, the tumor cells were strongly positive for vimentin but negative for keratin, macrophage ED1 antigen, alpha-smooth muscle actin, Factor VIII-related antigen, and S100 protein. Electron microscopy demonstrated the endothelial differentiation of the tumor cells, such as occasional luminal spaces, a small number of micropinocytotic vesicles, and interdigitating junctions with desmosomes between cell processes of adjacent cells. Furthermore, its endothelial origin was suggested by the presence of electron-dense rods resembling Weibel-Palade bodies. Instead of a definitive basement lamina surrounding the tumor cells, there were extracellular thin "anchoring filaments" that were attached to the cell surface at areas of increased electron density. These findings indicate that the tumor is of lymphatic vessel type rather than blood vessel type.
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After injury to the left foreleg, a 2 1/2-year-old Great Dane bitch developed severe oedema of the limb, which did not respond to pressure bandaging or a proteolytic agent. Skin breakdown occurred over the metacarpal region and lymph could be expressed from this lesion. The oedema spread, anaemia and dyspnoea developed and the animal died of respiratory insufficiency 55 days after the initial injury. Autopsy showed widespread infiltration of subcutis, fascial planes and some muscles of the leg by invasive cords and sheets of small endothelial-type cells which formed channels sometimes filled with blood. There was widespread diffuse metastatic neoplasm throughout the lung but no cavernous blood sinus formation. There were small metastases in local lymph nodes, kidney, bone marrow and spleen. It was concluded that the injury made obvious an oedema caused by pre-existing angiosarcoma.
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A malignant endothelial tumour arising in a congenitally lymphoedematous extremity is a sufficiently rare tumour to deserve the reporting of a new case. This tumour presented as small nodules on the back of the hand and in spite of midhumeral amputation widely scattered pulmonary metastases were present at the autopsy 28 months after the original presentation.
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