Composite hemangioendotheliomas have features of both benign and malignant vascular tumors. These tumors usually occur on or under the skin on the arms or legs. They may also occur on the head, neck, or chest. Composite hemangioendotheliomas are not likely to metastasize (spread) but they may come back in the same place. When the tumors metastasize, they usually spread to nearby lymph nodes.

Diagnostic Tests

See childhood vascular tumors for a description of tests and procedures used to diagnose composite hemangioendothelioma and find out whether the tumor has spread.

Treatment

Treatment of composite hemangioendotheliomas may include the following:

• Surgery to remove the tumor.
• Radiation therapy and chemotherapy for tumors that have spread.

Epithelioid hemangioendotheliomas can occur in children, but are most common in adults aged 30 to 50 years. They usually occur in the liver, lung, or in bone. They may be fast growing or slow growing. In about a third of cases, the tumor spreads to other parts of the body very quickly.

Signs and Symptoms

Signs and symptoms depend on where the tumor is in the body. Check with your child's doctor if your child has any of the following:

• On the skin, the tumors can be raised and rounded or flat, red-brown patches that feel warm.
• In the lung, there may be no early symptoms. Signs and symptoms that may occur include:
  • Chest pain.
  • Spitting up blood.
  • Anemia (weakness, feeling tired, or looking pale).
  • Trouble breathing (from scarred lung tissue).
• In bone, the tumors can cause breaks.

Tumors that occur in the liver or soft tissue may also cause signs and symptoms.

Diagnostic Tests

Epithelioid hemangioendotheliomas in the liver are found with CT scans and MRI scans. See childhood vascular tumors for a description of these tests and procedures used to diagnose epithelioid hemangioendothelioma and find out whether the tumor has spread. X-rays may also be done.

Treatment

Treatment of slow-growing epithelioid hemangioendotheliomas includes the following:

• Observation.

Treatment of fast-growing epithelioid hemangioendotheliomas may include the following:

• Surgery to remove the tumor when possible.
• Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that are likely to spread.
• Chemotherapy.
• Radiation therapy.
• Total hepatectomy and liver transplant when the tumor is in the liver.
• A clinical trial of targeted therapy (trametinib).

Kaposiform hemangioendotheliomas and tufted angiomas are blood vessel tumors that occur in infants or young children. These tumors can cause Kasabach-Merritt phenomenon, a condition in which the blood is not able to clot and serious bleeding may occur. In Kasabach-Merritt phenomenon, the tumor traps and destroys platelets (blood-clotting cells). Then there aren't enough platelets in the blood when needed to stop bleeding. This type of vascular tumor is not related to Kaposi sarcoma.

Signs and Symptoms

Kaposiform hemangioendotheliomas and tufted angiomas usually occur on the skin of the arms and legs, but may also form in deeper tissues, such as muscle or bone, or in the chest, abdomen, head, or neck.

Signs and symptoms may include the following:

• Firm, painful areas of skin that look bruised.
• Purple or brownish-red areas of skin.
• Pain with no visible lump.
• Easy bruising.
• Bleeding more than the usual amount from mucous membranes, wounds, and other tissues.

Patients who have kaposiform hemangioendothelioma and tufted angioma may have anemia (weakness, feeling tired, or looking pale).

Diagnostic Tests

See childhood vascular tumors for a description of tests and procedures used to diagnose kaposiform hemangioendothelioma and tufted angioma.

If a physical exam and MRI clearly show the tumor is a kaposiform hemangioendothelioma or a tufted angioma, a biopsy may not be needed. A biopsy is not always done because serious bleeding can occur.

Treatment

Treatment of kaposiform hemangioendotheliomas and tufted angiomas depends on the child's symptoms. Infection, delay in treatment, and surgery can cause bleeding that is life-threatening. Kaposiform hemangioendotheliomas and tufted angiomas are best treated by a vascular anomaly specialist.

Treatment for uncomplicated kaposiform hemangioendotheliomas and tufted angiomas may include the following:

• Observation.
• Surgery to remove the tumor.
• Laser surgery with a pulsed dye laser.
• Topical therapy (steroids or tacrolimus).
• Beta-blocker therapy (propranolol).
• Targeted therapy (sirolimus).

Treatment for complicated kaposiform hemangioendotheliomas and tufted angiomas may include the following:

• Chemotherapy, with or without steroid therapy.
• Targeted therapy (sirolimus), with or without steroid therapy.
• Surgery, with or without embolization.

Even with treatment, these tumors do not fully go away and can come back. Pain and inflammation may get worse with age, often around the time of puberty. Long-term effects include chronic pain, heart failure, bone problems, and lymphedema (the build up of lymph fluid in tissues).

Pseudomyogenic hemangioendothelioma can occur in children, but is most common in men aged 20 to 50 years. These tumors are rare, and usually occur on or under the skin, or in bone. They may spread to nearby tissue but usually do not spread to other parts of the body. In most cases, there are multiple tumors.

Signs and Symptoms

Pseudomyogenic hemangioendotheliomas may appear as a lump in soft tissue or may cause pain in the affected area.

Diagnostic Tests

See childhood vascular tumors for a description of tests and procedures used to diagnose pseudomyogenic hemangioendothelioma.

Treatment

Treatment of pseudomyogenic hemangioendotheliomas may include the following:

• Surgery to remove the tumor when possible. Amputation may be needed when there are multiple tumors in the bone.
• Chemotherapy.
• Targeted therapy (mTOR inhibitors).

Because pseudomyogenic hemangioendothelioma is so rare in children, treatment options are based on clinical trials in adults.

Retiform hemangioendotheliomas are slow growing, flat tumors that occur in young adults and sometimes children. These tumors usually occur on or under the skin of the arms, legs, and trunk. These tumors usually do not spread to other parts of the body.

Diagnostic Tests

See childhood vascular tumors for a description of tests and procedures used to diagnose retiform hemangioendothelioma.

Treatment

Treatment of retiform hemangioendotheliomas may include the following:

• Surgery to remove the tumor. Follow-up will include monitoring to see if the tumor comes back.
• Radiation therapy and chemotherapy when surgery cannot be done or when the tumor has come back.

Retiform hemangioendothelioma may come back after treatment.

Tests are used to diagnose childhood vascular tumors.

The following tests and procedures may be used:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps, lesions, or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. The picture can be printed to be looked at later.

• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

• X-ray: An x-ray of the bones or the chest. An x-ray is a type of energy beam that can go through the body onto film, making a picture of areas inside the body.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. A biopsy is not always needed to diagnose a vascular tumor.