Hemangioma. Questions & Answers. Dmitry V. Romanov

in the left half of the face.

      When segmental hemangioma is detected in a patient, it is necessary to exclude the presence of combined pathological syndromes, such as PHACE (s) and LUMBAR.

      1.9. What is hemangiomatosis?

      Hemangiomatosis is the presence of multiple (more than 4—5) hemangiomas on the skin (and/or mucous membrane) of a child. The number of hemangiomas in hemangiomatosis can vary from 5 to 1000 or more elements. In our practice, the maximum number of infantile hemangiomas detected in one child was 1096 elements, and there was damage not only to the skin, but also to visible mucous membranes (lips, oral mucosa, tongue, palate). The forms and types of hemangiomas in hemangiomatosis can be variable, but as a rule, these are small tumor-like formations, mainly up to 2—3 mm in diameter.

      There are two types of hemangiomatosis:

      – benign neonatal hemangiomatosis (only the skin is affected);

      – diffuse neonatal hemangiomatosis, in which, in addition to the skin, hemangiomas affect the parenchyma of the liver/spleen/intestine.

      Fig. 18 Benign neonatal hemangiomatosis. The child was diagnosed with 1096 hemangiomas on the skin and mucous membrane.

      Fig. 19 Benign neonatal hemangiomatosis. No treatment was performed, the child is 1.5 years old, hemangiomas are in the involution stage.

      1.10. What internal organs can be affected by hemangioma?

      Most often, pathological changes are detected in the largest parenchymatous organ of a person – in the liver. These pathological changes in a newborn are usually determined by a standard screening ultrasound examination. Detection of infantile hemangiomas of the spleen and kidneys in a newborn by ultrasound examination is extremely rare.

      There are three types of liver damage by infantile hemangioma: focal (28%), multiple (57%) and diffuse (15%).

      Fig. 20 Ultrasound examination of multiple hemangioma of the liver. Multiple round foci of reduced echogenicity are noted.

      Fig. 21 Focal hemangioma of the liver. CT data with contrast. MIP reconstruction of the image.

      Focal hemangioma of the liver (28%) is a rapidly involuting hemangioma that regresses immediately after birth. The incidence in boys and girls is the same. About 15% of children have infantile hemangiomas on their skin. More than 90% of the tumor volume decreases by 1.5—2 years of age.

      Multiple hemangioma of the liver (57%) is an infantile hemangioma that is very often accompanied by skin manifestations (77%). It is usually detected during liver screening examination in children with hemangiomatosis. This type of hemangioma occurs 2—3 times more often in girls than in boys. Since all infantile hemangiomas develop after birth, multiple hemangioma of the liver cannot be diagnosed prenatally by ultrasound examination. After involution of the infantile hemangioma of the liver, the parenchyma involved in the pathological process becomes normal.

      Diffuse hemangioma of the liver (15%) is an infantile hemangioma that is very often detected during the neonatal period. Diffuse hemangioma of the liver is not represented by normal liver tissue – the entire liver is replaced by a tumor. Half of the patients have skin hemangiomas. Girls are more often affected (in 70% of cases). With this change, liver function disorders are possible – liver failure.

      1.11. What is Kaposiform hemangioendothelioma (hemangioendothelioma)?

      Kaposiform hemangioendothelioma is a rare vascular tumor characterized by local aggression, but without metastasis. The incidence rate is approximately 1:100,000 children. This disease has no gender, boys and girls suffer from this disease with equal frequency. The head and neck are most often affected (40%), less often the trunk (30%) or limbs (30%). In the neonatal period, it occurs in 60% of cases, and is detected in 93% of cases during infancy.

      This diagnosis can also be made at an older age. Kaposiform hemangioendothelioma can occur in adults. The main method confirming the presence of Kaposiform hemangioendothelioma in a patient is an immunohistochemical study of removed pathological tissues.

      Fig. 22 Hemangioendothelioma in the right shoulder area.

      Fig. 23 Hemangioendothelioma in a newborn child in the left chest area.

      71% of patients with this vascular tumor have life-threatening Kasabach-Merritt syndrome.

      1.12. What is Kasabach-Merritt syndrome (phenomenon)?

      Kasabach-Merritt syndrome, also known as “hemangioma syndrome with thrombocytopenia”, most often manifests itself as the presence of Kaposiform hemangioendothelioma, which is complicated by thrombocytopenia (a significant decrease in the number of platelets), hemolytic anemia (destruction of red blood cells) and coagulopathy (impaired blood clotting). All these factors lead to severe bleeding. Kasabach-Merritt syndrome occurs equally often in boys and girls.

      Attempts to take histological material or perform surgery to remove hemangioendothelioma without first excluding Kasabach-Merritt syndrome lead to the development of life-threatening bleeding both during the operation and in the subsequent period!

      1.13. What vascular formation is most often confused with infantile hemangioma?

      The second most common vascular formation in pediatric practice is pyogenic granuloma. The primary distinguishing feature of pyogenic granuloma from infantile hemangioma is the appearance of a local round formation in children over 3 months of age.

      Conventionally, the third most common and most misdiagnosed nevus is Spitz’s nevus.

      1.14. What is pyogenic granuloma?

      Pyogenic granuloma is also called lobular capillary hemangioma. In most cases, acquired granuloma occurs, but cases of congenital pyogenic granuloma have also been described. A feature of pyogenic granuloma is rapid (eruptive, explosive) growth. This formation develops literally in a few days. The granuloma is a dense red papule that grows quickly and has a base in the form of a stalk (leg).

      Fig. 24 Pyogenic granuloma in the cheek area on the right. The patient is 2 years old.

      Fig. 25 Pyogenic granuloma in the back of the neck. The patient is 6 years old.

      Pyogenic granuloma is characterized by bleeding and ulceration. After bleeding and crust falling off, it may become smaller, but usually grows again, already with a larger base and a more dome-shaped raised part. Most often, pyogenic granuloma appears at the age of 3—7 years and only in 12% – in the first year of life. Pyogenic granuloma most often affects the skin (88.2%), less often – mucous membranes (11.8%). It occurs on the head and neck in 62% of cases, on the trunk – in 20%, on the upper limbs – in 13%, on the lower limbs – in 5%.

      Patients have a history of microtraumas of the skin at the site of formation of pyogenic granuloma: cracks in the skin, allergies and dermatitis, scratching, insect bites (mosquitoes).

      1.15. What is Spitz naevus?

      Spitz naevus is a relatively rare benign type of skin tumor. Spitz naevus is part of a group of skin tumors commonly