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1.1. What is the current classification of vascular anomalies?

The main working classification for specialists in the field of vascular pathology is the classification of the International Society for the Study of Vascular Anomalies (ISSVA) (Table 1). The latest changes to the Classification were made in April 2018.

Ternovsky S.D.

In 1962, Soviet surgeons Ternovsky S.D. and Kondrashin N.I. proposed dividing vascular tumors and vascular anomalies into 2 main groups of pathological changes, and only 20 years later, in 1982, Mulliken and Glowacki proposed a similar division, confirming the assumptions of Russian researchers with immunohistochemical studies.

1.2. What is a cavernous hemangioma?

The word “cavern” comes from the Latin word “caverna” – a cave, a cavity. In medicine, caverns were defined as pathological cavities that arise in the body as a result of partial tissue necrosis followed by its decay and rejection of the dead mass. The question arises, what does hemangiomas have to do with it? The term “cavernous hemangioma” appeared in the early 19th century and implied pathologically dilated vessels in the form of cavities of various sizes and shapes. Due to the lack of any other clarifying research methods (at that time there was only microscopy and histology), this term was retained in subsequent years, was included in classifications and was used to designate a number of pathological conditions characterized by tumor-like proliferation of vessels, most of which were dilated and had vascular cavities on the section. After the advent of immunohistochemistry, this term lost its significance, as it became possible to accurately determine the tissue nature of these pathological conditions. Today, many cavernous hemangiomas turn out to be venous or arteriovenous malformations.

1.3. What is a capillary hemangioma?

The term capillary hemangioma appeared during the period of using the term “cavernous hemangioma”, based on histological studies, due to the prevalence of pathological capillaries in the tissues under study, all infantile hemangiomas at that time were considered capillary hemangiomas and in some cases continue to be considered (especially in the conclusions of pathologists). At present, this term has lost its meaning.

1.4. What are the types of hemangiomas?

Infantile hemangiomas are divided into the following types according to the prevalence of the pathological process:

– superficial (the tumor-like formation spreads superficially and affects all layers of the dermis);

– deep (the tumor-like formation is located subcutaneously, in most cases there are no vascular manifestations on the surface of the skin, or isolated telangiectasias are observed);

– combined (a combination of superficial and deep pathological changes).

Fig. 5 Superficial infantile hemangioma.

Fig. 6 Combined infantile hemangioma.

Fig. 7 Deep infantile hemangioma.

According to the area of damage, infantile hemangiomas are divided into:

– focal (damage to one anatomical area);

– multifocal (damage to several anatomically unrelated areas);

– segmental (damage to several adjacent anatomical areas at once).

Fig. 8 Multifocal infantile hemangioma.

Fig. 9 Segmental infantile hemangioma.

1.5. What is a congenital hemangioma?

A congenital hemangioma differs from an infantile hemangioma not only in that it is laid down in utero, but also in the intrauterine onset of the development cycle and formation of pathological volumetric vascular tissue. At the time of birth, a congenital hemangioma has its maximum size; no growth of the hemangioma after birth is observed. Some specialists mistakenly classify congenital hemangiomas as infantile, assuming that they began their tumor-like development in utero, and by the time of birth they have completed the proliferation phase (active growth), but immunohistochemical and genetic studies have shown that these tumor-like formations have different natures and cannot be combined into one term “infantile hemangioma”. After birth, congenital hemangiomas can involute on their own, partially or completely, but in some cases, congenital hemangiomas remain unchanged over the following years.

Fig. 10 Congenital hemangioma in a 1 month old child. Clinically, it is preliminarily assessed as a NICH hemangioma.

Fig. 11 Congenital infantile hemangioma in a 3-day-old child. Clinically, it is preliminarily assessed as a RICH hemangioma.

1.6. What types of congenital hemangioma are there?

Congenital hemangiomas are divided into:

– rapidly involuting congenital hemangioma (RICH);

– non-involuting congenital hemangioma (NICH);

– partially involuting congenital hemangioma (PICH).

1.7. What is the difference between congenital hemangiomas?

The most similar clinical picture when examining a patient are RICH and PICH hemangiomas. It is not for nothing that 5 years ago only 2 types of congenital hemangiomas were determined RICH and NICH and only recent studies have revealed the PICH variant. The tumor-like formation does not have smooth contours and may also rise unevenly above the surface of the skin.

Fig. 12 NICH hemangioma in the thigh area.

Fig. 13 NICH hemangioma in the occipital region.

Hemangiomas are capable of rapid involution during the first months from the moment of birth in cases with RICH – complete disappearance with a deficit of subcutaneous fat in this place subsequently, and in the case of PICH with partial involution of pathological tissue, usually more than 50—60% of the volume.

NICH hemangiomas are characterized by the appearance of a low cylinder (like a “washer”), protruding quite evenly above the surface of the skin, dense upon palpation, on the surface of which venulo- and telangiectasias are determined; the classic variant of infantile hemangiomas – red papules – are not determined on the surface of the tumor. After birth, the tumor remains unchanged (NICH hemangioma).

Fig. 14 NICH hemangioma in the cheek area. The patient is 1 month old.

Fig. 15 The same patient after 7 years.

All types of these hemangiomas are not sensitive to the administration of beta-blockers.

1.8. What is a segmental hemangioma?

The term “segmental lesion” came to this area of medicine from maxillofacial surgery and implies the involvement of several anatomical areas in the pathological process at once (for example, the forehead, orbit, cheek, or, for example, the hand and forearm).

Fig. 16 Combined segmental infantile hemangioma of the left half of the face.

Fig. 17 Segmental infantile hemangioma 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 called moles. Spitz nevi initially grow rapidly, forming pink or brown bumps on the surface of the skin.

They are usually found on the head, neck, or legs of children and young adults. The tumor is named after Dr. Sophie Spitz, a pathologist who first described these nevi.

Fig. 26 Spitz nevus on the forearm.

Fig. 27 Dermoscopy data from the same patient.

Sophie Spitz

Sophie Spitz (February 4, 1910 – August 11, 1956) was an American pathologist who published the first case series of “juvenile melanoma” (a special form of benignmelanocytic nevi), skin lesions that became known as Spitz nevi.. For her contributions to pathology, and especially her foresight in advocating the use of the Pap test, she is recognized as the preeminent pathologist of her time.