Vascular tumors are growths of blood vessels. The majority of vascular tumors in children are benign (harmless), and malignant vascular tumors are extremely rare. The most common vascular tumor of infancy is the infantile hemangioma. Other notable types of vascular tumors are described below.
Vascular anomalies can be difficult to accurately diagnose and treat. Our team ensures the best possible outcome by combining the expertise of specialists and tailoring treatment for each patient.
Congenital hemangiomas are benign vascular tumors that are made up of small thin-walled blood vessels in the skin. They appear as either flat or raised masses that are red to purple in color with a surrounding pale rim and usually feel warm to touch. Congenital hemangiomas can be present anywhere on the skin, but occasionally they can be found in the liver as well.
Unlike infantile hemangiomas, congenital hemangiomas are fully developed at the time of birth. Occasionally, these tumors are seen on a prenatal ultrasound. After birth, some congenital hemangiomas shrink very rapidly over the first year of life, while others persist throughout life. Therefore, congenital hemangiomas are usually further classified as:
- Non-involuting congenital hemangiomas (NICH), which remain throughout life without going away
- Rapidly involuting congenital hemangiomas (RICH), which rapidly shrink after birth
- Partially involuting congenital hemangiomas (PICH), which shrink partially after birth
The majority of congenital hemangiomas do not cause any problems. In some cases, the skin may break down (ulcerate), causing pain and/or bleeding. This is usually treated with wound care, including an ointment such as petrolatum ointment to protect the skin, a prescribed antibiotic ointment and a dressing. Very large congenital hemangiomas may rarely lead to heart problems due to the large amount of blood flow through the tumor.
Congenital hemangiomas are usually diagnosed clinically and typically do not require any additional testing. Sometimes, imaging may be required. A biopsy is rarely needed but can be helpful when the diagnosis is unclear.
The majority of congenital hemangiomas do not require treatment, and watchful waiting is the most appropriate management. Unlike infantile hemangiomas, propranolol therapy does not tend to be effective for congenital hemangiomas. Surgical excision may be indicated for some lesions, particularly those that either do not go away or only go away partially. In addition, surgical excision can help remove residual tissue after involution if needed.
Pyogenic granulomas are benign small bumps on the skin caused by a growth of small blood vessels. These growths can occur on the skin or mucous membranes and at any age, though they generally are not present at birth or in very young babies. The lesions appear red to reddish brown, and can be present anywhere on the body. The cause of these growths is unknown, but for many patients, there is a history of trauma to the area.
Pyogenic granulomas tend to bleed due to their fragile surface, however, the amount of blood loss is rarely threatening. If bleeding occurs, firm manual pressure should be applied to stop the bleeding.
A diagnosis is usually made clinically. When the diagnosis is unclear, a skin biopsy can be performed.
Surgical shaving or excision is the preferred method of treatment for pyogenic granulomas.
Tufted angiomas are rare benign vascular tumors of the blood vessels that grow in the skin and subcutaneous tissue. Tufted angiomas mainly affect infants and young children, although older children and adults can get them as well. The lesion appears at birth in approximately 50% of cases; otherwise, it typically begins within the first year of life. Tufted angiomas appear as red, purple or brown patches on the skin. The skin over the tumor may appear hairier than the surrounding areas. They can be small or large in their size, and they may thicken and darken over time. Tufted angiomas can appear on any part of the body but are most common on the arms, legs and trunk.
Tufted angiomas are associated with a bleeding disorder called Kasabach-Merritt phenomenon (KMP) in approximately 10% of patients. KMP results in decreased platelet counts and decreased ability to form blood clots. Therefore, KMP can be life-threatening and requires treatment. A sudden increase in the mass size may indicate KMP.
Diagnosis is usually confirmed with a skin biopsy. Blood tests are checked to evaluate platelet level and clotting function.
Small tufted angiomas and those that are not causing symptoms or complications may not need treatment. For lesions that are causing pain and/or are large, treatment options may include:
- Low-dose aspirin, which could help prevent blood clots
- Sirolimus, an immune-suppressing medication that helps to decrease the growth of the blood vessels
- Pulsed dye laser to lighten the color of the lesion
- Surgical excision to remove the tumor
Children with Kasabach-Merritt Phenomenon will likely require hospitalization for treatment. In addition to the options above, treatment for children with KMP may include:
- Chemotherapeutic agents
- Blood transfusions
- Clotting agents
Kaposiform Hemangioendothelioma (KHE)
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically occurs during infancy and early childhood. KHE presents as a firm mass in the skin with a deep red or purple color. They are usually warm to touch. KHE most commonly occurs on the extremities, head, neck and abdomen. Notably, KHE may invade surrounding tissue and extend deep into muscle, bone and other structures.
Like tufted angiomas, the most serious potential complication of KHE is Kasabach-Merritt phenomenon (KMP), a bleeding disorder where platelet levels and clotting factors are low due to trapping within the tumor. A sudden increase in size of the mass may indicate KMP.
Additional symptoms depend on where the KHE is located, as various structures such as muscle, bone or nerve may be affected.
Diagnosis is usually confirmed with a skin biopsy. Blood tests are used to evaluate platelet level and clotting function. An ultrasound and/or MRI will usually be conducted to determine size.
Treatment for KHE varies depending on each child’s symptoms, extent of the disease and presence of KMP. Treatment may include:
- Chemotherapeutic agents
- Blood transfusions
- Clotting agents
- Surgery in cases where the tumor can be safely removed