Hemangioendothelioma (angiosarcoma): causes, symptoms, diagnosis, treatment

Hemangioendothelioma (syn.: angiosarcoma) is a malignant tumor originating from the endothelial elements of blood and lymphatic vessels. W. F. Lever and O. Sehaurnburg-Lever (1983) distinguish two types of this tumor: angiosarcoma, developing on the head and face in elderly individuals, and secondary angiosarcoma, occurring in chronic lymphatic edema (Stewart-Treves syndrome).

The first type of hemangioendothelioma is clinically manifested mostly by one lesion, which is a node of various sizes of irregular shape, livid or deep red color, located on the scalp or linden. Small daughter nodes appear on the periphery. The tumor quickly increases, disintegrates, metastasizes early, first to the cervical lymph nodes, then to the lungs and liver.

Pathomorphology of hemangioendothelioma (angiosarcoma). The differences depend on the degree of differentiation of the tumor elements. In the peripheral parts of the tumor, they are more differentiated and form vascular structures, often anastomosing with each other, lined with one or more layers of large endothelial markers, most often cubic in shape. In the center of the tumor, infiltrating growth with barely noticeable vascular slits is noted, the cells here are less differentiated, large, atypical, sometimes protrude in the form of papillae into the lumen of the vessel and completely close it. Sometimes solid strands of spindle-shaped cells are located between the collagen fibers. When impregnated with silver nitrate, basal membranes of the vessels are visible, and in solid areas - a dense network of reticulin fibers. The tumor stroma is infiltrated with extravasates from erythrocytes and lymphocytes. Electron microscopy of polygonal and spindle-shaped tumor cells revealed that they were surrounded by aggregates of fibrous substances resembling collagen fibers. The nuclei of tumor cells are located centrally, contain a lot of euchromatin, nucleoli and nuclear bodies, in the cytoplasm there are a small number of mitochondria, polyribosomes and expanded cisterns of the endoplasmic reticulum, microfilaments and dense bodies in the perinuclear zone. Lysosomes are found, and micropinocytosis vesicles are determined in the peripheral parts of the cells.

Type II hemangioendotheliomas (Stewart-Treves syndrome) develop in the area of chronic lymphostasis after radical mastectomy. The tumor manifests itself as cutaneous and subcutaneous nodules that develop several years after radical mastectomy in the edematous tissue of the upper limb on the side of the operation. The skin nodules are bluish in color, their number and size quickly increase. They can ulcerate. They most often metastasize to the lungs.

The pathomorphology of hemangioendothelioma (angiosarcoma) is similar to that of idiopathic hemangioendothelioma, but is distinguished by more pronounced infiltrating growth with the location of tumor elements linearly between bundles of collagen fibers.

Histogenesis. This tumor is called lymphangiosarcoma due to its specific localization (lymphostasis). However, the presence of erythrocytes in the tumor and the formation of not only lymphatic but also blood vessels, as well as pericytes, a positive reaction to acid phosphatase in atypical endotheliocytes give reason to believe that its genesis is associated with the endothelium of both lymphatic and blood vessels.

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