Kaposiform hemangioendothelioma, or KHE, is a type of tumor that grows in the blood vessels. Blood vessels help move blood around the body. This tumor usually grows just underneath the skin, but it can also grow deeper inside the chest or abdomen.

This type of tumor grows quickly, but it is benign, which means it is not cancer and does not spread to other parts of the body.

KHE is so rare that there is little data on how many people have it. Most patients diagnosed with KHE are either born with it or are diagnosed before they are one year old.

It is not yet known what causes KHE to form. Scientists are always working to understand how tumors form, but it can be hard to prove.

No, KHE is not known to run in families.

Patients with KHE typically have a tumor that can be seen under the skin. It looks purple, puffy, and is warm to the touch. Other symptoms include pain and difficulty moving the area of the body with the tumor.

Lab tests: If your child has symptoms of KHE, doctors will order lab tests of your child’s blood to check the levels of different kinds of blood cells. It’s important to check the platelet count, which can be low in patients with KHE and leads to increased bleeding.

Imaging: If your child has symptoms of KHE, doctors will use imaging scans such as ultrasound, CT, and MRI to look at where the tumor is and how big it is.

Biopsy: To check if the tumor is KHE your child’s doctor will do a biopsy, taking a small sample from the tumor with a needle. An expert, called a pathologist, will study cells from the sample under the microscope to see what kind of tumor it is.

Treatment for each patient will be unique. You should go to an expert in vascular tumors to decide the best approach for your child’s tumor. You can contact MyPART for help finding experts near you.

Treatment options to discuss with your doctor include:

Watch and wait: In some cases, the tumor may be small and not harming other parts of the body, or it may go away on its own. In this case it may be safest for your child’s doctor to check the tumor regularly without treating it.

Surgery: In cases where the tumor can be completely removed or growing very quickly, surgery to remove the tumor may be the best treatment.

Chemotherapy: Chemotherapy drugs have been successfully used to treat KHE.

Steroids: Steroids are drugs that can be given to treat the tumor, but they do not work for all cases of KHE.

Embolization therapy: Depending on where the KHE is, embolization therapy can be used. This type of therapy cuts off the blood supply to the tumor. This causes the tumor cells to die because they can’t get oxygen and nutrients.

The estimate of how a disease will affect you in the long term is called prognosis. Every person is different, and prognosis will depend on many factors, such as:

• Where the tumor is in the body
• How much of the tumor was taken out during surgery

If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis.

Doctors estimate KHE survival rates by how groups of people with KHE have done in the past. Because there are so few KHE patients, these rates may not be very accurate. When KHE is found and treated when it is small, prognosis is good and most patients survive.