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

Gemangioendothelioma (syn: angiosarcoma) is a malignant tumor that originates from the endothelial elements of the blood and lymphatic vessels. WF Lever and O. Sehaurnburg-Lever (1983) distinguish two types of this tumor: angiosarcoma developing on the head and face in the elderly, and angiosarcoma secondary, occurring in chronic lymphatic edema (Stewart-Trivs syndrome).

The first type of hemangiendothelioma is clinically manifested mostly by a single lesion, representing a different size of a node of irregular shape, livid or saturated red, located on the scalp or lime. On the periphery appear small daughter nodules. The tumor quickly increases, it breaks up, early metastasizes, first into the cervical lymph nodes, then into the lungs and liver.

Pathomorphology of hemangiendothelioma (angiosarcoma). Differences depend on the degree of differentiation of tumor elements. In the peripheral parts of the tumor they are more differentiated and constitute vascular structures, often anastomosing with each other, lined with one or more layers of large endothelial tags, more often a cubic form. In the center of the tumor there is infiltrating growth with barely noticeable vascular gaps, the cells here are less differentiated, large, atypical, sometimes protruding in the form of papillae into the lumen of the vessel and completely closing it. Sometimes between the collagen fibers there are solid strands of spindle-shaped cells. When impregnating with silver nitrate, the basal membrane of the vessels is visible, and in the 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 are surrounded by aggregates of fibrous substances resembling collagen fibers. The nuclei of tumor cells are centrally located, contain a lot of euchromatin, nucleoli and nuclear bodies; in the cytoplasm there are a small number of mitochondria, polyribosomes and expanded endoplasmic reticulum cages, microfilaments and dense bodies in the perinuclear zone. Lysosomes are found, and micropinocytosis vesicles are detected in the peripheral parts of the cells.

Hemangioendothelioma of the second type (Stewart-Trivs syndrome) develops in the area of chronic lymphostasis after radical mastectomy. The tumor is manifested by cutaneous and subcutaneous nodules developing several years after radical mastectomy in the edematous tissue of the upper limb on the side of the operation. Skin nodules of cyanotic color, their number and sizes increase rapidly. Can ulcerate. Metastasis most often in the lungs.

The pathomorphology of hemangiendothelioma (angiosarcoma) is analogous to that of idiopathic hemangiendothelioma, it has a more pronounced infiltrating growth with the location of tumor elements linearly between bundles of collagen fibers.

Histogenesis. This tumor in connection with a certain localization (lymphostasis) is called lymphangiosarcoma. 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 acidic phosphatase in atypical endotheliocytes suggests that its genesis is associated with the endothelium of both lymphatic and blood vessels.

[1], [2], [3], [4], [5], [6], [7]