Lymphangioendothelioblastoma

Lymphangioendothelial sarcoma is a rare tumor of endothelial cells of lymphatic and blood vessels, characterized by a favorable outcome with radical removal along with all enlarged lymph nodes of regional metastases. It mainly occurs in adulthood and old age, and has a predominantly malignant course. Features of involutive changes in morphostructure suggest the origin of lymphangoendothelial sarcomas from perivascular tissues (Paget's). The course of the disease is characterized by rapid tumor growth with the appearance of masses and their metastasis. These manifestations are caused by infiltration of the tumor and nodes of adjacent tissues and swelling accompanying the formation of cavities in lymph and angiomassive tumors. They are important pathogenetic features of lymphangioendeliosarcomas. Parallelism is characteristic between the activity of the tumor process and the degree of involution; the accompanying changes are characteristic exclusively of peripheral nodes. Character

**Lymphangioendothelial blastoma (LEB)** is a rare disease characterized by the malignant growth of malignant cells of lymphangioid endothelial or mesenchymal elements of the skin or soft tissues. LEB is a rare variant of soft tissue sarcoma of the limangiosarcoma type. This neoplasm has the most favorable prognosis among malignant soft tissue tumors.

Oncological diseases of soft tissues: rare cases and examples from real life of patients

“There are very few people in Russia who live more than 5 years after being diagnosed with recurrent cancer”

**(Alexandra Obolentseva, Moscow)

**Lymphatic vessels** are part of the vascular system responsible for the transport of lymph in the body. Together with the walls of the vessels, the tissue that these vessels form is called lymphatic endothelium.

According to the WHO definition, **lymphangioma** is a benign tumor that develops from mesenchymal derivatives in the form of either smooth capillaries, trabeculae, or spirally convoluted, complexly articulated blood and lymphatic vessels with characteristic peristaltic movements. These tumors can be congenital or develop during life. Main localizations: soft tissues of the head and neck, face, shoulder, upper limb, abdominal wall and lower leg. May be multiple. Despite the softness of the structure, it can be complicated by bleeding and lead to the death of patients. Treatment is surgical, including possible replacement of the defect with lymphatic vessels.

Lymphangiomas form in most cases in the first two years of a child’s life; the peak incidence occurs at 3-4 months and 9 months. In most cases - 80% of cases, all this occurs in newborn children. This is due to an increase in the activity of one of the hormones - prolactin, with an increase in which a more rapid and pronounced formation of lymphangiomas is observed.