Hemangioma. Questions & Answers. Dmitriy Romanov

is Kasabakh-Merritt syndrome (phenomenon)?

      Kasabakh-Merritt syndrome, also known as hemangioma thrombocytopenia syndrome, is mostly evident by the presence of a large vascular formation (the most common formation is a Kaposiform hemangioendothelioma but there may be others), which is complicated by thrombocytopenia (platelet count reduction), гемолитической hemolytic anemia (red blood cell destruction) as well as coagulopathy (clotting problem). All these factors lead to the severe bleeding. The Kasabach-Merritt syndrome is equally common in boys and girls.

      Kasabakh-Merritt syndrome. Kaposiform hemangioendothelioma.

      The same patient a general view.

      1.10. What is pyogenic granuloma?

      The pyogenic granuloma is also called lobular capillary hemangioma. In most cases, acquired granuloma is presented but there are also cases of congenital pyogenic granuloma described. It is basically solid red papule that grows rapidly and usually forms a stalk (pedicle).

      Pyogenic granuloma in the area of the nasal slope.

      The pyogenic granuloma is characterized by bleeding (64,2%) and ulceration (36,3%) tendency. It can decrease after the bleeding and crust falling off but usually it grows over again. It appears at the age of 6—7 years most commonly but in 12,4% of cases it appears on the first year of life. The pyogenic granuloma mostly affects skin (88,2%), less often the mucous membranes (11,8%). It is found on the head and neck in 62%, body is affected in 20%, upper limbs in 13%, lower limbs in 5% of cases.

      Pyogenic granuloma. Bleeding from granulomas.

      25% of patients had a history of injury at the pyogenic granuloma location or skin changes (such as a capillary or arteriovenous malformation). The modern theory suggests that pyogenic granuloma inducts secondary reactive skin lesions after injury.

      1.11. What else is considered to be vascular tumors?

      Angiosarcoma is a tumor characterized by malignancy. It is identified in less than 1% of cases amongst soft tissue sarcomas. Angiosarcoma is extremely rare in children (approximately 1%). It is equally often detected in boys and girls. The average age of diagnosis is 12 months of life.

      Cutaneovisceral angiomatosis with thrombocytopenia. This disease is also called multifocal lymphangioendotheliomatosis with thrombocytopenia. Multifocal vascular lesion affects skin and gastrointestinal tract, accompanied by platelet count reduction (thrombocytopenia). Lungs, bones, liver, spleen and muscles are also affected.

      Intramuscular Ensinger hemangioma (Ensinger hemangioma is a small vascular type or intramuscular hemangioma – capillary type) is a vascular tumor that occurs in the skeletal muscle. In occurs in children in 25% of cases. The average age of tumor detecting is 25 years.

      Epithelioid hemangioendothelioma is a malignant endothelial tumor with an unpredictable clinical course. In occurs in children in 7% of cases. This tumor is usually multifocal and affects skin, bones, liver, lungs (lesion might be bilateral).

      Infant myofibroma (term “infant hemangiopericytoma” was used earlier). This is the most common fibroid tumor in infants. There are three forms of infant myofibroma: solitary (common), multifocal (infant myofibromatosis), widespread (internal organs are affected). Up to 60% of cases are detected at birth. The tumor can grow during early childhood. It is detected more often in boys than in girls (1,6:1). It looks like an infant hemangioma very often, which requires differential diagnosis.

      Tufted angioma, or Nakagawa angioblastoma. This tumor is clinically and microscopically similar to Kaposiform hemangioendothelioma but it is less aggressive. It can show local invasive growth but does not metastasize. Tufted angioma manifests itself during infancy and early childhood. Just like Kaposiform hemangioendothelioma, tufted angioma can be associated with Kasabach-Merritt syndrome.

      II. Causes of infantile hemangioma appearance, family background

      2.1. What is the cause for a hemanhioma to appear?

      Infantile hemangioma appears at 6—10^th weeks of pregnancy, when damage of tissue (vessels) rudiment occurs. There are ca. 10 theories of infantile hemangioma development (fissural, neurological, traumatic, fetal, hypoxic etc). Among current theories we can call theory of circulating endothelial precursor cells, placental theory (placenta damage during pregnancy time) etc. Currently, however, there is no generally accepted and confirmed theory, which could reliably explain the appearance of infantile hemangiomas.

      3D ultrasound reconstruction of the fetus at 10 weeks.

      Possible causes of hemangiomas:

      – amniocentesis, chorionic biopsy;

      – mother’s age;

      – mother’s level of education;

      – multiple pregnancy;

      – first labor;

      – placenta previa;

      – preeclampsia;

      – placenta abnormalities (retroplacental hematoma, placental infarction, dilated vascular communications);

      – placental hypoxia;

      – if mother took erythropoietin and/or fertile drugs.

      According to our and foreign studies, the cause for the infant hemangioma development is a viral disease of a pregnant woman (possibly, virus infection carrier state and/or contact with a viral patient) during the period from 6 to 10 weeks of pregnancy (first trimester). As a result of the virus penetration into the pregnant woman blood, the placental barrier gets damaged, which leads to the migration of placenta cells into the fetal skin. This theory is confirmed by the studies that have revealed that infant hemangioma cells are immunohistochemically identical to the placenta cells. Pathological cell masses occur in areas with a low blood flow and activate after the baby’s birth only (through hormonal release) and begin to form a pathological blood-vascular structure.