Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that may appear at birth or shortly after, often presenting with a bluish or purple lesion and tight skin. Led by Dr. Waner and Dr. O, the Vascular Birthmark Institute specializes in diagnosing and treating KHE to help children achieve healthy outcomes.
What is Kaposiform Hemangioendothelioma?
Kaposiform hemangioendothelioma (KHE) is a rare, benign vascular tumor that typically develops at birth or within the first few days of life. Although noncancerous, KHE can cause serious health risks, including organ damage and bleeding disorders like Kasabach-Merritt phenomenon (KMP). The tumor often appears as a firm, bluish or purple area on the skin, commonly affecting the abdomen, head, neck, or limbs. Our doctors use advanced diagnostic tools and individualized treatment plans to manage KHE effectively and minimize potential complications for our young patients.
Signs & Symptoms of Kaposiform Hemangioendothelioma:
- Firm, bluish or purple discoloration of the skin
- Tightened, stretched skin over the affected area
- Swelling in the tumor region
- Lesion most commonly located on the abdomen, head, neck, or limbs
- Rapid growth of the tumor after birth
- Pain or tenderness in the affected area
- Risk of bleeding or clotting abnormalities (if associated with Kasabach-Merritt phenomenon)
- Potential signs of organ dysfunction if internal organs are involved
Treatments for Kaposiform Hemangioendothelioma
At the Vascular Birthmark Institute, we understand that no two cases of kaposiform hemangioendothelioma (KHE) are alike. When you bring your child to us, we begin with a comprehensive evaluation, which may include a physical examination, blood tests, MRI imaging, and sometimes a biopsy. Once we confirm the diagnosis, our doctors design a treatment plan tailored to your child’s specific needs.
Vincristine Therapy
Vincristine is a chemotherapy medication that is commonly used to treat KHE. Although it is traditionally known for treating cancer, in the case of KHE, vincristine works by slowing down the growth of the abnormal blood vessels that make up the tumor. It is usually administered through a central line directly into the bloodstream once a week. Over time, this medication can shrink the tumor and reduce the risk of complications like Kasabach-Merritt phenomenon (KMP).
Steroid Therapy
Steroid medications can also help treat KHE. If your child’s tumor is still relatively small or not causing significant symptoms, we may recommend a short course of steroids. Steroids help by reducing inflammation and suppressing the abnormal cell growth that fuels the tumor. However, because steroids can have side effects, especially in young children, our doctors use them carefully and typically for a limited period, balancing the benefits with any potential risks.
Surgery
In certain aggressive or complicated cases, surgery may be recommended to remove the tumor. Surgical treatment is typically reserved for tumors that do not respond to medical therapy or are causing health problems, such as severe bleeding or organ dysfunction. Our surgeons aim to remove as much of the tumor as possible while preserving the function of surrounding tissues. If surgery is needed, we will guide you through every step, from pre-operative planning to recovery, to ensure the best possible results for your child.
Schedule Your Consultation
Vascular Birthmark Institute is a leading center for the diagnosis and treatment of rare vascular tumors like kaposiform hemangioendothelioma. Led by Dr. Waner and Dr. O, our team offers expert, compassionate care tailored to your child’s needs. We combine advanced diagnostics with customized treatment plans to ensure the best possible outcomes. If your child has been diagnosed with or is suspected to have kaposiform hemangioendothelioma, we encourage you to schedule a consultation with our experienced doctors today.
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