Kaposiform hemangioendothelioma (KHE) is a non-malignant tumor of the blood vessels which develops below the skin in the majority of cases. However, in rare cases KHE can develop in the organs, close to the lungs or within the abdominal cavity. Children are at most risk of developing the rare form of kaposiform hemangioendothelioma. Another condition, KasselbachMerritt syndrome (KMS), can sometimes result from a hemangioendothelioma.
Although these vascular tumors are not malignant, the development of KMS is a serious complication that will cause rapid tumor growth. Another complication of KMS is a significant reduction in platelet numbers, which in turn inhibits the blood’s ability to clot. When clots do form, there is the risk of a blockage of normal blood flow reaching tissues.
Symptoms and Diagnosis
A tumor that is present at birth or develops before the child reaches the age of one may indicate a KHE. Tumors are raised in appearance and look similar to bruised skin. If the area around the tumor sweats more than expected or there is excessive hair, a KHE is the most likely cause. You will typically notice KHEs around the arms and legs. However, they can also develop on the torso, head or neck. There are also visible and physical signs of KMS, which include pain as the tumor grows and purple to brown appearance due to bleeding that is occurring beneath the skin.
Kaposiform Hemangioendothelioma Treatment
Medications are the first course of treatment that is recommended in less severe cases of KHE. Aspirin can help reduce clotting, sirolimus is used to improve clotting and reduce the size of tumors and platelet therapy is often recommended where complications exist. Ultimately, your child’s treatment plan will depend on a number of factors which Dr. Greg Levitin will identify during the evaluation stage of treatment.
The Vascular Birthmark Center can offer surgical options when KHE tumors are safe to remove. Dr. Greg Levitin will also closely monitor your child’s KHE, especially during the first vital year of life when complications from Kasselbach-Merritt syndrome are most likely to arise. All treatment options are fully explained in further detail during your appointment with a specialist at the Vascular Birthmark Center. It is important to Dr. Greg Levitin that you are able to make informed decisions about your child’s health and aftercare.
If you have been given a tentative diagnosis of KHE for your child and would like a second opinion or input from a specialist, reach out to the Vascular Birthmark Center in either New York or Los Angeles. We are available over the phone or via our website where you can receive a free consultation where most, if not all, your questions will be answered in real time.