Hemangioma. Questions & Answers. Dmitry V. Romanov

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