Hemangioma. Questions & Answers. Dmitriy Romanov
of the lower leg.
Combined infantile hemangioma in the parietal region. Focal form.
Сombined infantile hemangioma in the shoulder area. Focal form.
Also, infantile hemangiomas are divided according to skin lesions range:
– focal;
– multifocal;
– segmental.
Segmental infantile hemangioma in the right half of the face.
Combined infantile hemangioma in the chest area. The multifocal form.
Segmental infantile hemangioma in the chest and right shoulder.
Congenital hemangiomas are divided into:
– rapidly involuting congenital hemangioma – RICH;
– non-involuting congenital hemangioma – NICH;
– partially involuting congenital hemangioma – PICH.
Rapidly involuting congenital hemangioma – RICH.
Partially involuting congenital hemangioma – PICH.
Non-involuting congenital hemangioma – NICH.
Clinical example. Non-involuting congenital hemangiom.
Non-involuting congenital hemangiom. Newborn.
The same patient. 6 y.o.
1.4. What is the difference between a congenital hemangioma and an infant one?
A congenital hemangioma appears and grows in utero and has its largest size at birth. It does not grow after birth anymore and can decrease and disappear or remain the same size even without treatment.
An infant hemangioma appears basically during first month of baby’s life. In rare cases, a baby is born with it. An active growth of hemangioma begins during first weeks of baby’s life. An active growth phase lasts up to 5—6 months and can last up to 9—12 months. Then, a plateau phase or growth stabilization begins, after that an involution period begins (reverese development of hemangioma) which can take years.
1.5. What is a segmental hemangioma?
If an infant hemangioma affects several anatomical areas at once (e.g., forehead, orbit, cheek and nose on one side of the face or, for example, the wrist and forearm), it is called a segmental hemangioma. In most cases, a segmental hemangioma is located at skin level, e.g. it is superficial.
Why is it so important to separate this nosology? A large damage area can lead to ulceration and bleeding. In addition, segmental infant hemangiomas cab be a signal for certain syndorms presence, such as PHACE (s) and LUMBAR.
Segmental infantile hemangioma in the left half of the face.
Segmental infant hemangioma in the lower half of the trunk and limbs.
1.6. What is hemangiomatosis?
Hemangiomatosis is the appearance of multiple (more than 4—5) small (from 1—2 mm) hemangiomas on the baby’s skin. The number of hemangiomas in hemangiomatosis varies from 5 to 1000 elements on the skin and mucous membranes. Such hemangiomas are located on the surface of the skin, but sometimes there is a combination of small superficial hemangiomas with combined ones.
Hemangiomatosis is of two types:
– benign neonatal hemangiomatosis;
– diffuse neonatal hemangiomatosis. Hemagiomas are present not only on skin but also in liver/spleen/intestines in this form of hemangiomatosis.
Benign neonatal hemangiomatosis. 1000 Infantile hemangiomas.
Benign neonatal hemangiomatosis. Proliferative phase.
Benign neonatal hemangiomatosis. Involutive phase.
1.7. What internal organs can be affected by a hemangioma?
Out of all the internal organs, hemangioma most often affects the liver. Three types of liver damage are distinguished: focal (28%), multiple (57%) and diffuse (15%).
Focal liver hemangioma (28%) is presented by a rapid involuting congenital hemangioma (RICH), which regresses right after birth. The occurrence rate in boys and girls is the same. About 15% of children have infant hemangiomas on their skin. More than 90% of tumor size decreases to the 13th month of life.
Multiple liver hemangioma (57%) is an infant hemangioma, which is often accompanied by skin manifestations (77%). It is usually identified during liver screening in children with hemangiomatosis (four to five or more skin infant hemangiomas). It is 2—3 times more often in girls than in boys. Since all infant hemangiomas develop after birth, multiple liver hemangiomas cannot be diagnosed prenatally. After regression of infant hemangioma, liver parenchyma that is involved in the pathological process (functionally active epithelial cells) becomes normal.
Focal liver hemangioma.
Multiple liver hemangioma.
Diffuse liver hemangioma (15%) is an infant hemangioma which is very often detected during the neonatal period. Diffuse liver hemagioma is not presented by the normal liver tissue, the entire liver is replaced by a tumor. Half of the patients have skin hemangiomas in this case. Girls suffer more often (in 70% of cases).
Diffuse liver hemangioma.
1.8. What is Kaposiform hemangioendothelioma (hemangioendothelioma)?
Kaposiform hemangioendothelioma is a a rare vascular tumor, that is characterized by local aggression, but without metastasis process. The occurrence rate is approximately 1: 100,000 children. It occurs with the same frequency in boys and girls. The head and neck (40%) are more often affected, less often the body (30%) or limbs (30%). It occurs in 60% of cases in the neonatal period and is identified in 93% of cases during infancy. This diagnosis can be made at an older age. Kaposiform hemangioendothelioma can occur in adults.
In 71% of patients with this tumor, life-threatening Kasabakh-Merritt syndrome is identified.
In some cases, differential diagnostic procedure with infantile hemangioma is required.
Kaposiform