Kaposiform hemangioendothelioma

The first step in treating your child is forming an accurate and complete diagnosis. It’s important to understand that neither you nor (probably) your child’s regular pediatrician will be able to diagnose kaposiform hemangioendothelioma (KHE) by simply looking at the lesion.

There’s also a related, milder tumor called tufted angioma that looks very much like KHE. Only experienced vascular anomalies specialists will be able to tell the difference between KHE and tufted angioma and other vascular anomalies.

When should I consult a vascular anomalies specialist?

We advise consulting a vascular anomalies specialist in three situations:

• if your child has a vascular skin lesion and a low platelet count
• if your child has a vascular skin lesion that seems to be growing beyond expectations
• if your child has a vascular skin lesion that gets larger, darker and more painful after a platelet transfusion

How is KHE diagnosed?

When you make an appointment at Children’s Hospital Boston, we start by requesting all outside imaging, labs, notes and photographs to begin preparation for your visit. Your visit includes a complete medical history and thorough physical exam.

We will review outside imaging studies and will not repeat studies unless necessary. After a discussion between you and your doctor, your child will have one or more of the following imaging tests, which can help determine the correct diagnosis:

• Magnetic resonance imaging (MRI) — This high-resolution scan shows how large your child’s tumor is as well as its relationship to nearby muscles, nerves, bones and other blood vessels.
• Ultrasound (also called ultrasonography) — An ultrasound also shows the size of the tumor and allows your doctor to see how much blood is flowing through it.
• Computerized tomography scan (also called a CT or CAT scan) – This test is only occasionally used for KHE. A CT scan shows detailed images the area around your child’s tumor, including the bones, muscles, fat and organs. CT scans are more detailed than general x-rays, but do involve radiation exposure.
• Biopsy — If the results of the physical exam and imaging tests are not conclusive, your doctor may take a biopsy of your child’s lesion. A biopsy is a simple surgical procedure where a doctor removes a small tissue sample.
• Examining the tumor's cellular appearance under a microscope allows the physician to determine definitively whether your child has KHE.
• If your child has had a biopsy done elsewhere, we can often use it instead of performing another biopsy.
• Complete blood count (CBC) — If your child’s doctor suspects KHE, he or she will order a complete blood count to check your child’s platelet level. Other blood studies may also be helpful.

After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.