Endothelioma is a rare neoplasm originating from the inner lining of blood or lymphatic vessels. It can occur in any organs and tissues, but more often affects the skin, subcutaneous fat, nasopharynx, bones, testicles and ovaries. It is a rounded node. The color of the node ranges from grayish to rich red. Depending on the type of growth, the endothelioma may be benign or malignant. Malignant tumors are characterized by slow infiltrative growth and rarely metastasize. The diagnosis is made taking into account the examination data and histological examination. Treatment – surgery, radiotherapy, chemotherapy.
An endothelioma is a tumor whose source is flat cells of mesenchymal origin lining the inner surface of lymphatic and blood vessels. Previously, the group of endotheliomas included a large number of neoplasms of a similar structure, localized in the meninges, pericardium, peritoneum, pleura, salivary glands, etc. Subsequently, scientists found that some of these tumors originate from the neuroepithelium, mesothelium or epithelium of the salivary glands.
The exclusion of a significant number of neoplasms from the group of endotheliomas led to the fact that some specialists began to doubt the validity of such a diagnosis. Subsequently, the researchers were able to confirm the nature of endotheliomas, currently this group includes a small number of rare tumors. The disease is included in the WHO classification. Endothelioma treatment is carried out by specialists in the field of oncology, otolaryngology, dermatology, orthopedics and other areas (depending on the localization of the neoplasm).
Tumors originating from the endothelium of lymphatic vessels are called lymphoendotheliomas, from blood vessels – hemangioendotheliomas. Both types of tumors can be both benign and malignant. Benign endotheliomas are characterized by noninvasive growth and the absence of metastases. Malignant neoplasms also grow quite slowly, but their growth has an infiltrative character. Metastasis of endotheliomas is rarely observed. When the process spreads, the lungs are usually affected.
Benign endotheliomas look like a plexus of endothelial cell masses separated by connective tissue partitions. There are also neoplasms resembling angiomas. Such endotheliomas consist of many branching and intertwining lymphatic or blood vessels of various diameters.
The vascular epithelium differs from the usual flat cells of the endothelium and is a tall cylindrical cells. In the lumen of the vessels of hemangioendotheliomas contains blood, in the lumen of the vessels of lymphoendotheliomas – lymphatic fluid. Rarely there are endotheliomas that are located inside a large blood vessel and partially or completely block its lumen, playing the role of a blood clot.
Malignant endotheliomas are very rarely diagnosed. Histological examination of malignant hemangioendothelioma reveals alternating sections of alveolar and solid structure. Atypical cells of oval or rounded shape with large nuclei are detected. There is a large number of mitoses.
Malignant lymphoendothelioma consists of a large number of cavities of various sizes and shapes. The inner shell of the cavities is represented by large cells forming areas of solid tissue and papillary structures. The diagnosis of endothelioma based on the study of a histological sample is associated with certain difficulties, the use of special dyes may be required.
Benign endotheliomas originating from blood vessels are usually located in the skin (often affect the skin of the face), are small round-shaped nodes of pink, red or burgundy color. With minor injuries, endotheliomas are easily damaged and bleed. Ulceration is possible. Palpation is painless. The general condition of the patient is not disturbed. The tumor does not progress or slowly increases in size without germinating the surrounding tissue. It does not pose a threat to the patient’s life.
Malignant endotheliomas originating from blood vessels are more often localized in the skin and nasopharynx, sometimes affecting bones and internal organs. When located in the skin area, a tumor-like formation of a dense consistency is detected. The color of the endothelioma ranges from pink to dark red, depending on the amount of blood in the cavities and vessels of the tumor. In the early stages, an asymptomatic course is characteristic, in the subsequent clinical picture is determined by the germination of nearby tissues and the location of distant metastases.
When endothelioma is localized in the nasopharynx area, patients note difficulties in nasal breathing. Headaches and increased fatigue are possible. The tumor causes frequent nosebleeds, anemia may develop. With the further growth of endothelioma, there is an obturation of the nasal cavity with the development of nasal and hearing loss, impaired taste and sense of smell. With metastasis, there are violations of the function of the corresponding organs.
Malignant endotheliomas originating from lymphatic vessels are located in the skin, bones, testicles, ovaries and other organs. It is possible to damage the breast area a few years after a radical mastectomy. Like hemangioendothelioma, lymphoendothelioma is a dense node of a rounded or oval shape, while the endothelioma usually has a grayish or whitish color, rather than red or pink, due to the absence of blood in the tumor cavities. The symptoms depend on the prevalence of the process and the presence or absence of distant metastases.
The diagnosis is established on the basis of an external examination and additional research data. The list of studies depends on the localization of the endothelioma. When located in the nasopharynx, rhinoscopy is performed, CT or MRI of the skull is prescribed. When internal organs are affected, CT or MRI of the abdominal cavity or chest is performed.
With endotheliomas in the bone area, radiography or scintigraphy is performed. If distant metastasis is suspected, patients are referred for chest radiography. The final diagnosis is made taking into account the histological examination data.
The treatment is surgical. The endothelioma is excised within healthy tissues. In case of malignant tumors, lymphadenectomy is performed, X-ray therapy is used in the postoperative period. According to the results of recent studies, a good effect is observed when prescribing sarcolysin. When using chemotherapy drugs, drug therapy is first performed, after the endothelioma is reduced, surgery is performed, then a course of chemotherapy and radiotherapy is prescribed.