Childhood Vascular Tumors Treatment (PDQ®): Treatment - Patient Information [NCI]

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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.

General Information About Childhood Vascular Tumors

Childhood vascular tumors form from cells that make blood vessels or lymph vessels.

Vascular tumors can form from abnormal blood vessel or lymph vessel cells anywhere in the body. They may be benign (not cancer) or malignant (cancer). There are many types of vascular tumors. The most common type of childhood vascular tumor is hemangioma, which is a benign tumor that usually goes away on its own.

Because malignant vascular tumors are rare in children, there is not a lot of information about what treatment works best.

Tests are used to detect (find) and diagnose childhood vascular tumors.

The following tests and procedures may be used:

  • Physical exam and 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.
    Abdominal ultrasound; drawing shows a child lying on an exam table during an abdominal ultrasound procedure. A technician is shown pressing a transducer (a device that makes sound waves that bounce off tissues inside the body) against the skin of the abdomen. A computer screen shows a sonogram (picture).
    Abdominal ultrasound. An ultrasound transducer connected to a computer is pressed against the skin of the abdomen. The transducer bounces sound waves off internal organs and tissues to make echoes that form a sonogram (computer picture).
  • 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.
    Computed tomography (CT) scan of the abdomen; drawing shows a child lying on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
    Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
  • 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).
    Magnetic resonance imaging (MRI) of the abdomen; drawing shows a child lying on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child's abdomen helps make the pictures clearer.
    Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child's abdomen helps make the pictures clearer.
  • 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.

Childhood vascular tumors may be classified into four groups.

Benign tumors

Benign tumors are not cancer. This summary has information about the following benign vascular tumors:

  • Infantile hemangioma.
  • Congenital hemangioma.
  • Benign vascular tumors of the liver.
  • Spindle cell hemangioma.
  • Epithelioid hemangioma.
  • Pyogenic granuloma (lobular capillary hemangioma).
  • Angiofibroma.
  • Juvenile nasopharyngeal angiofibroma.

Intermediate (locally aggressive) tumors

Intermediate tumors that are locally aggressive often spread to the area around the tumor. This summary has information about the following locally aggressive vascular tumors:

  • Kaposiform hemangioendothelioma and tufted angioma.

Intermediate (rarely metastasizing) tumors

Intermediate (rarely metastasizing) tumors sometimes spread to other parts of the body. This summary has information about the following vascular tumors that rarely metastasize:

  • Retiform hemangioendothelioma.
  • Papillary intralymphatic angioendothelioma.
  • Composite hemangioendothelioma.
  • Kaposi sarcoma.

Malignant tumors

Malignant tumors are cancer. This summary has information about the following malignant vascular tumors:

  • Epithelioid hemangioendothelioma.
  • Angiosarcoma of soft tissue.

Treatment Option Overview

There are different types of treatment for childhood vascular tumors.

Different types of treatment are available for children with vascular tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Because vascular tumors in children are rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with childhood vascular tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include the following specialists:

  • Pediatric vascular anomaly specialist (expert in treating children with vascular tumors).
  • Pediatric surgeon.
  • Orthopedic surgeon.
  • Radiation oncologist.
  • Pediatric nurse specialist.
  • Rehabilitation specialist.
  • Psychologist.
  • Social worker.

Some treatments cause side effects months or years after treatment has ended.

Some treatments, such as chemotherapy and radiation therapy, cause side effects that continue or appear months or years after treatment has ended. These are called late effects. Late effects of treatment may include:

  • Physical problems.
  • Changes in mood, feelings, thinking, learning, or memory.
  • Second cancers (new types of cancer).

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information).

Eleven types of standard treatment are used:

Beta-blocker therapy

Beta-blockers are drugs that decrease blood pressure and heart rate. When used in patients with vascular tumors, beta-blockers may help shrink the tumors. Beta-blocker therapy may be given by vein (IV), by mouth, or placed on the skin (topical). The way the beta-blocker therapy is given depends on the type of vascular tumor and where the tumor first formed.

The beta-blocker propranolol is usually the first treatment for hemangiomas. Infants treated with IV propranolol may need to have their treatment started in a hospital. Propranolol is also used to treat benign vascular tumor of liver and kaposiform hemangioendothelioma.

Other beta-blockers used to treat vascular tumors include atenolol, nadolol, and timolol.

Infantile hemangioma may also be treated with propranolol and steroid therapy or propranolol and topical beta-blocker therapy.

See the drug information summary on Propranolol Hydrochloride for more information.

Surgery

The following types of surgery may be used to remove many types of vascular tumors:

  • Excision: Surgery to remove the entire tumor and some of the healthy tissue around it.
  • Laser surgery: A surgical procedure that uses a laser beam (a narrow beam of intense light) as a knife to make bloodless cuts in tissue or to remove a skin lesion such as a tumor. Surgery with a pulsed dye laser may be used for some hemangiomas. This type of laser uses a beam of light that targets blood vessels in the skin. The light is changed into heat and the blood vessels are destroyed without damaging nearby skin.
  • Total hepatectomy and liver transplant: A surgical procedure to remove the entire liver followed by a transplant of a healthy liver from a donor.
  • Curettage: A procedure in which abnormal tissue is removed using a small, spoon-shaped instrument called a curette.

The type of surgery used depends on the type of vascular tumor and where the tumor formed in the body.

For malignant tumors, even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the tumor will come back, is called adjuvant therapy.

Photocoagulation

Photocoagulation is the use of an intense beam of light, such as a laser, to seal off blood vessels or destroy tissue. It is used to treat pyogenic granuloma.

Embolization

Embolization is a procedure that uses particles, such as tiny gelatin sponges or beads, to block blood vessels in the liver. It may be used to treat some benign vascular tumors of the liver and kaposiform hemangioendothelioma.

Chemotherapy

Chemotherapy is a treatment that uses drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect tumor cells in those areas (regional chemotherapy). Chemotherapy for hemangiomas may also be given topically (applied to the skin in a cream or lotion).

The way the chemotherapy is given depends on the type of the vascular tumor being treated.

Sclerotherapy

Sclerotherapy is a treatment used to destroy the blood vessel with the tumor. A liquid is injected into the blood vessel, causing it to scar and break down. Over time, the destroyed blood vessel is absorbed into normal tissue. The blood flows through nearby healthy veins instead. Sclerotherapy is used in the treatment of epithelioid hemangioma.

Radiation therapy

Radiation therapy is a treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or keep them from growing. There are two types of radiation therapy:

  • External radiation therapy uses a machine outside the body to send radiation toward the tumor.
  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the tumor.

The way the radiation therapy is given depends on the type of the vascular tumor being treated. External radiation is used to treat some vascular tumors.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to attack specific tumor cells. Targeted therapies usually cause less harm to normal cells than chemotherapy or radiation therapy do. Angiogenesis inhibitors are a type of targeted therapy.

  • Angiogenesis inhibitors are drugs that stop cells from dividing and prevent the growth of new blood vessels that tumors need to grow. The targeted therapy drugs thalidomide, sorafenib, pazopanib, sirolimus, and bevacizumab are angiogenesis inhibitors used to treat childhood vascular tumors.

Immunotherapy

Immunotherapy is a treatment that uses the patient's immune system to fight disease. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against disease.

Interferon is a type of immunotherapy used to treat childhood vascular tumors. It interferes with the division of tumor cells and can slow tumor growth. It is used in the treatment of juvenile nasopharyngeal angiofibroma, kaposiform hemangioendothelioma, and epithelioid hemangioendothelioma.

Other drug therapy

Other drugs used to treat childhood vascular tumors or manage their effects include the following:

  • Steroid therapy: Steroids are hormones made naturally in the body. They can also be made in a laboratory and used as drugs. Steroid drugs help shrink some vascular tumors.
  • Non-steroidal anti-inflammatory drugs (NSAIDs): NSAIDs are commonly used to decrease fever, swelling, pain, and redness. Examples of NSAIDs are aspirin, ibuprofen, and naproxen. In the treatment of vascular tumors, NSAIDs can increase the flow of blood through the tumors and decrease the chance that an unwanted blood clot will form.
  • Antifibrinolytic therapy: These drugs help the blood clot in patients who have Kasabach-Merritt syndrome. Fibrin is the main protein in a blood clot that helps stop bleeding and heal wounds. Some vascular tumors cause fibrin to break down and the patient's blood does not clot normally, causing uncontrolled bleeding. Antifibrinolytics help prevent the breakdown of fibrin.

Observation

Observation is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change.

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the research process. Clinical trials are done to find out if new treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way disease will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose tumors have not gotten better. There are also clinical trials that test new ways to stop tumors from recurring (coming back) or reduce the side effects of treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the vascular tumor may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your child's condition has changed or if the tumor has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Benign Tumors

Infantile Hemangioma

Infantile hemangiomas are the most common type of benign vascular tumor in children. An infantile hemangioma may also be called a "strawberry mark." Immature cells that are meant to form blood vessels form a tumor instead. These tumors are not usually seen at birth but appear when the infant is 3 to 6 weeks old. Most hemangiomas get bigger for about 5 months, then stop growing and slowly fade away completely during the next several years. It is rare for them to come back.

Hemangiomas may be on the skin, in the tissue below the skin, and/or in an organ. They are usually on the head and neck but can be anywhere on or in the body. Hemangiomas may appear as a single lesion, one or more lesions spread over a larger area of the body, or multiple lesions in more than one part of the body. Lesions that are spread over a larger area of the body or multiple lesions are more likely to cause problems.

Risk Factors

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesn't mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk.

Infantile hemangiomas are more common in the following:

  • Girls.
  • Whites.
  • Premature babies.
  • Twins, triplets, or other multiple births.
  • Babies of mothers who are older at time of the pregnancy or who have problems with the placenta during pregnancy.

Other risk factors for infantile hemangiomas include the following:

  • Having certain syndromes.
    • PHACE syndrome: A syndrome in which the hemangioma spreads across a large area of the body (usually the head or face). Other health problems involving the large blood vessels, heart, eyes, and/or brain may also occur.
    • LUMBAR/PELVIS/SACRAL syndrome: A syndrome in which the hemangioma spreads across a large area of the lower back. Other health problems that affect the urinary system, genitals, rectum, anus, brain, spinal cord, and nerve function may also occur.

Having more than one hemangioma or an airway hemangioma increases the risk of having other health problems.

  • Multiple hemangiomas: Having more than five hemangiomas on the skin is a sign that there may be hemangiomas in an organ, most commonly the liver. Heart, muscle, and thyroid gland problems can also occur.
  • Airway hemangiomas: Hemangiomas in the airway usually occur along with a large, beard-shaped area of hemangioma on the face (from the ears, around the mouth, lower chin, and front of neck). It is important for airway hemangiomas to be treated before the child has trouble breathing.

Signs and Symptoms

Infantile hemangiomas may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

  • Skin lesions: An area of spidery veins or lightened or discolored skin may appear before the hemangioma does. Hemangiomas occur as firm, warm, bright red-blue lesions on the skin. Lesions that form ulcers are also painful. Later, as the hemangiomas go away, they begin fading in the center before flattening and losing color.
  • Lesions below the skin: Lesions that grow under the skin in the fat may appear blue or purple. If the lesions are deep enough under the skin surface, they may not be seen.
  • Lesions in an organ: There may be no signs that hemangiomas have formed on an organ.

Although most infantile hemangiomas are nothing to worry about, if your child develops any lumps or red or blue marks on the skin check with your child's doctor. He or she can recommend a specialist if needed.

Diagnostic Tests

A physical exam and history are usually all that are needed to diagnose infantile hemangiomas. If there is something about the tumor that looks unusual, a biopsy may be done. If the hemangioma is deeper inside the body with no change to the skin, or the lesions are spread across a large area of the body, an ultrasound or MRI may be done. See the General Information section for a description of these tests and procedures.

If the hemangiomas are part of a syndrome, more tests may be done such as an echocardiogram, magnetic resonance angiogram, and eye exam.

Treatment

Most hemangiomas fade and shrink without treatment. If the hemangioma is large or causing other health problems, treatment may include the following:

  • Propranolol or other beta-blocker therapy.
  • Topical beta-blocker therapy for hemangiomas that are in one area of the skin.
  • Steroid therapy, before beta-blocker therapy is begun or when beta-blockers cannot be used.
  • Pulsed dye laser surgery, for hemangiomas that have ulcers or have not gone away.
  • Surgery (excision) for hemangiomas that have ulcers, cause vision problems, or have not gone away.
  • Combined therapy, such as propranolol and steroid therapy or propranolol and topical beta-blocker therapy.

Congenital Hemangioma

Congenital hemangioma is a benign vascular tumor that begins forming before birth and is fully formed when the baby is born. They're usually on the skin but can be in another organ.

There are three types of congenital hemangiomas:

  • Rapidly Involuting Congenital Hemangioma: These tumors go away on their own 12 to 15 months after birth. They can form ulcers, bleed, and cause temporary heart and blood clotting problems. The skin may look a little different even after the hemangiomas go away.
  • Partial Involuting Congenital Hemangioma: These tumors do not go away completely.
  • Non-Involuting Congenital Hemangioma: These tumors never go away on their own.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose congenital hemangioma.

Treatment

Treatment of rapidly involuting congenital hemangioma and partial involuting congenital hemangioma may include the following:

  • Observation only.

Treatment of non-involuting congenital hemangioma may include the following:

  • Surgery to remove the tumor depending on where it is and whether it is causing symptoms.

Benign Vascular Tumors of the Liver

Benign vascular tumors of the liver may be focal (a single lesion in one area of the liver), multifocal (multiple lesions in one area of the liver), or diffuse (multiple lesions in more than one area of the liver).

The liver has many functions, including filtering blood and making proteins needed for blood clotting. Sometimes, blood that normally flows through the liver is blocked or slowed by the tumor. This sends blood directly to the heart without going through the liver and is called a liver shunt. This can cause heart failure and problems with blood clotting.

Focal Tumors

Focal tumors are usually rapidly involuting congenital hemangiomas or non-involuting congenital hemangiomas.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose focal benign vascular tumors.

Treatment

Treatment of focal tumors of the liver depends on whether symptoms occur and may include the following:

  • Observation.
  • Drugs to manage symptoms, including heart failure and blood clotting problems.
  • Embolization of the liver to manage symptoms, including heart failure.

Multifocal and Diffuse Tumors

Multifocal and diffuse tumors of the liver are usually infantile hemangiomas. Diffuse tumors of the liver can cause serious effects, including problems with the thyroid gland and heart. The liver can enlarge, press on other organs, and cause more symptoms.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose multifocal or diffuse benign vascular tumors.

Treatment

Treatment of multifocal and diffuse liver tumors may include the following:

  • Observation for multifocal tumors of the liver that do not cause symptoms.
  • Beta-blocker therapy (propranolol).
  • Chemotherapy.
  • Steroid therapy.
  • Total hepatectomy and liver transplant, when the tumors do not respond to drug therapy.

If a vascular tumor of the liver does not respond to standard treatments, a biopsy may be done to see if the tumor has become malignant.

Spindle Cell Hemangioma

Spindle cell hemangiomas contain cells called spindle cells. Under a microscope, spindle cells look long and slender.

Risk Factors

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesn't mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk. Spindle cell hemangiomas are likely to occur in children with the following syndromes:

  • Maffucci syndrome, which affects cartilage and skin.
  • Klippel-Trenaunay syndrome, which affects blood vessels, soft tissues, and bones.

Signs

Spindle cell hemangiomas appear on or under the skin. They are painful red-brown or bluish lesions that usually appear on the arms or legs. They can begin as one lesion and develop into more lesions over years.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose spindle cell hemangioma.

Treatment

There is no standard treatment for spindle cell hemangiomas. Treatment may include the following:

  • Surgery to remove the tumor.

Spindle cell hemangiomas may come back after surgery.

Epithelioid Hemangioma

Epithelioid hemangiomas usually form on or in the skin, especially the head, but can occur in other areas, such as bone.

Signs and Symptoms

Epithelioid hemangiomas are sometimes caused by injury. On the skin, they may appear as firm pink to red bumps and may be itchy. Epithelioid hemangioma of the bone may cause swelling, pain, and weakened bone in the affected area.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose epithelioid hemangioma.

Treatment

There is no standard treatment for epithelioid hemangiomas. Treatment may include the following:

  • Surgery (curettage or resection).
  • Sclerotherapy.
  • Radiation therapy in rare cases.

Epithelioid hemangiomas often come back after treatment.

Pyogenic Granuloma

Pyogenic granuloma is also called lobular capillary hemangioma. It is most common in older children and young adults but may occur at any age.

The lesions are sometimes caused by injury or from the use of certain medicines, including birth control pills and retinoids. They may also form for no known reason inside capillaries (the smallest blood vessels) or other places on the body. Usually there is only one lesion, but sometimes multiple lesions occur in the same area or the lesions may spread to other areas of the body.

Signs

Pyogenic granulomas are raised, bright red lesions that may be small or large and smooth or bumpy. They grow quickly over weeks to months and may bleed a lot.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose pyogenic granuloma.

Treatment

Some pyogenic granulomas go away without treatment. Other pyogenic granulomas need treatment that may include the following:

  • Surgery (excision or curettage) to remove the lesion.
  • Laser photocoagulation.

Pyogenic granulomas often come back after treatment.

Angiofibroma

Angiofibromas are rare. They are benign skin lesions that usually occur with a condition called tuberous sclerosis (an inherited disorder that causes skin lesions, seizures, and mental disabilities).

Signs

Angiofibromas appear as red bumps on the face.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose angiofibroma.

Treatment

Treatment of angiofibromas may include the following:

  • Surgery (excision) to remove the tumor.
  • Laser therapy.
  • Targeted therapy (sirolimus).

Juvenile Nasopharyngeal Angiofibroma

Juvenile nasopharyngeal angiofibromas are benign tumors but they can invade nearby tissue. They begin in the nasal cavity and may spread to the nasopharynx, the paranasal sinuses, the bone around the eyes, and sometimes to the brain.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose juvenile nasopharyngeal angiofibroma.

Treatment

Treatment of juvenile nasopharyngeal angiofibromas may include the following:

  • Surgery (excision) to remove the tumor.
  • Radiation therapy.
  • Chemotherapy.
  • Immunotherapy (interferon).
  • Targeted therapy (sirolimus).

Intermediate Tumors that Spread Locally

Kaposiform Hemangioendothelioma and Tufted Angioma

Kaposiform hemangioendotheliomas and tufted angiomas are blood vessel tumors that occur in infants or during early childhood. 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. Signs and symptoms may include the following:

  • Firm, painful areas of skin that look bruised.
  • Purple or brownish-red areas of skin.
  • Easy bruising.
  • Bleeding more than the usual amount from mucous membranes, wounds, and other tissues.
  • Anemia (weakness, feeling tired, or looking pale).

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose kaposiform hemangioendothelioma.

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 and supportive care to manage bleeding may include the following:

  • Steroid therapy which may be followed by chemotherapy.
  • Non-steroidal anti-inflammatory drugs (NSAID), such as aspirin.
  • Immunotherapy (interferon).
  • Antifibrinolytic therapy to improve blood clotting.
  • Chemotherapy with one or more anticancer drugs.
  • Beta-blocker therapy (propranolol).
  • Surgery (excision) to remove the tumor, with or without embolization.
  • Targeted therapy (sirolimus).
  • A clinical trial of targeted therapy (sirolimus) and steroid therapy.

Even with treatment, these tumors do not fully go away and can come back. Long-term effects include chronic pain, heart failure, bone problems, and lymphedema (the build up of lymph fluid in tissues).

Intermediate Tumors that Rarely Spread

Retiform Hemangioendothelioma

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 often come back after treatment, but they usually do not spread to other parts of the body.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose retiform hemangioendothelioma.

Treatment

Treatment of retiform hemangioendotheliomas may include the following:

  • Surgery (excision) 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.

Papillary Intralymphatic Angioendothelioma

Papillary intralymphatic angioendotheliomas are also called Dabska tumors. These tumors form in or under the skin anywhere on the body. The tumors contain channels that look like lymph vessels. Lymph nodes are sometimes affected.

Signs

Papillary intralymphatic angioendotheliomas may appear as firm, raised, purplish bumps, which may be small or large. These tumors grow slowly over time.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose papillary intralymphatic angioendothelioma.

Treatment

Treatment of papillary intralymphatic angioendotheliomas may include the following:

  • Surgery (excision) to remove the tumor.

Composite Hemangioendothelioma

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 the General Information section 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.

Kaposi Sarcoma

Kaposi sarcoma is a cancer that causes lesions to grow in the skin; the mucous membranes lining the mouth, nose, and throat; lymph nodes; or other organs. It is caused by the Kaposi sarcoma herpes virus (KSHV). In the United States, it usually occurs in people who have a weak immune system caused by AIDS or by drugs used in organ transplants. It is very rare in children and can be caused by HIV infection and rare immune system disorders.

Signs

Signs in children may include the following:

  • Lesions in the skin, mouth, or throat. Skin lesions are red, purple, or brown and change from flat, to raised, to scaly areas called plaques, to nodules.
  • Swollen lymph nodes.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose Kaposi sarcoma.

Treatment

Treatment of Kaposi sarcoma may include the following:

  • Chemotherapy.

See the PDQ summary on Kaposi Sarcoma Treatment for information about Kaposi sarcoma in adults.

Malignant Tumors

Epithelioid Hemangioendothelioma

Epithelioid hemangioendotheliomas can occur in children, but are most common in adults between 30 and 50 years of age. They usually occur in the liver, lung, or in bone. They may be either 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:

  • 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 occur may 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.

Diagnostic Tests

Epithelioid hemangioendotheliomas in the liver are found with CT scans and MRI scans. See the General Information section 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.
  • Total hepatectomy and liver transplant when the tumor is in the liver.

Angiosarcoma of the Soft Tissue

Angiosarcomas are fast-growing tumors that form in blood vessels or lymph vessels in any part of the body, usually in soft tissue. Most angiosarcomas are in or near the skin. Those in deeper soft tissue can form in the liver, spleen, and lung.

These tumors are very rare in children. Children sometimes have more than one tumor in the skin and/or liver.

Risk Factors

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get the disease; not having risk factors doesn't mean that you will not get the disease. Talk with your child's doctor if you think your child may be at risk. Risk factors for angiosarcomas include the following:

  • Being exposed to radiation.
  • Chronic (long-term) lymphedema, a condition in which extra lymph fluid builds up in tissues and causes swelling.
  • Having a benign vascular tumor. A benign tumor, such as a hemangioma, may become an angiosarcoma but this rare.

Signs

Signs of angiosarcoma depend on where the tumor is and may include the following:

  • Red patches on the skin that bleed easily.
  • Purple tumors.

Diagnostic Tests

See the General Information section for a description of tests and procedures used to diagnose angiosarcoma and find out whether the tumor has spread.

Treatment

Treatment of angiosarcoma may include the following:

  • Surgery to completely remove the tumor.
  • A combination of surgery, chemotherapy, and radiation therapy for angiosarcomas that have spread.
  • Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that began as infantile hemangiomas.
  • A clinical trial of targeted therapy, radiation therapy, and surgery with or without chemotherapy.

To Learn More About Childhood Vascular Tumors

For more information about childhood vascular tumors, see the following:

  • Soft Tissue Sarcoma Home Page
  • Computed Tomography (CT) Scans and Cancer
  • Targeted Cancer Therapies

For more childhood cancer information and other general cancer resources, see the following:

  • About Cancer
  • Childhood Cancers
  • CureSearch for Children's Cancer
  • Late Effects of Treatment for Childhood Cancer
  • Adolescents and Young Adults with Cancer
  • Children with Cancer: A Guide for Parents
  • Cancer in Children and Adolescents
  • Staging
  • Coping with Cancer
  • Questions to Ask Your Doctor about Cancer
  • For Survivors and Caregivers

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.

PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government's center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of childhood vascular tumors. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change.

The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Clinical trials are listed in PDQ and can be found online at NCI's website. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237).

Permission to Use This Summary

PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary]."

The best way to cite this PDQ summary is:

PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Vascular Tumors Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-vascular-tumors-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 27253005]

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Last Revised: 2017-03-01


If you want to know more about cancer and how it is treated, or if you wish to know about clinical trials for your type of cancer, you can call the NCI's Cancer Information Service at 1-800-422-6237, toll free. A trained information specialist can talk with you and answer your questions.