What is Kaposiform Hemangioendothelioma?
Rarely, vascular tumors can behave more like other soft tissue tumors, in that they can infiltrate tissues and create problems. The symptoms can be quite serious and will require a vascular anomalies expert to intervene… sometimes urgently.
of a child’s shoulder.
Kaposiform hemangioendothelioma (KHE) is one of these types of tumors. Fortunately, it’s benign and does not spread. In many ways, it follows the natural course of other infantile hemangiomas with a growth phase followed by gradual involution. It is frequently misdiagnosed as a hemangioma.
Nonetheless, several potential problems can occur: KHE can cause a depletion of platelets and clotting factors in the blood, causing a serious condition know as Kasabach-Merritt phenomenon. In this situation, normal clotting of blood does not occur, leaving the patient vulnerable to serious bleeding consequences.
- Along with impairment of clotting, a diffuse skin rash can occur, known as petechiae.
- Pain is a common complaint, since KHE can infiltrate muscle, joints, and sometimes, bone.
- Swelling can sometimes be quite severe. The area involved is usually firm to the touch.
- Mobility of the involved tissues or extremities can be severely limited.
The diagnosis is typically made with an examination and biopsy. Once the correct diagnosis is made, monitoring of the tumor is required. Specifically, blood coagulation needs to be checked routinely, and drops in platelet or clotting factor levels will need to be remedied with specific medications. Several medications have been used effectively to treat KHE. Steroids have been used traditionally. However, newer medications have shown good efficacy, including vincristine (chemotherapy agent) and sirolimus (immunosuppressant used in transplant patients).
With close attention and initiation of medical therapy, KHE can be eradicated. The role for surgery and embolization has diminished with advancements in medical therapy.
Why choose Dr Panossian
- He received his medical education at Tufts University School of Medicine.
- Graduated at the top of his class at UCLA, receiving Phi Beta Kappa and Summa Cum Laude honors.
- Was accepted into an elite combined general surgery and plastic surgery residency at the Keck School of Medicine of USC.
- Completed subspecialty training in craniofacial surgery at the Hospital for Sick Children in Toronto and Harvard Medical School.
- Was mentored by Dr. Ron Zuker in the practice of facial paralysis reconstruction. This prestigious fellowship position was available to only one surgeon in the United States.
- Is affiliated with various charitable and educational organizations, including Operation Smile and Mending Kids.
- Is a member of the American Society of Plastic Surgeons and the highly selective American Association of Plastic Surgeons, reserved for only a select group of individuals nationally who have demonstrated excellence in academic plastic surgery.
- Holds memberships in several other professional societies including the American College of Surgeons and the American Society of Reconstructive Microsurgery.
- He serves on the Board of Directors for Mending Kids and the Gondobay Manga Foundation, a non-profit organization dedicated to the improvement of lives in Sierra Leone.
- Has been nominated by his peers annually since 2012 as a “Super Doctor.”
- Served as an expert medical consultant and appeared on The Doctors, Grey’s Anatomy, and Nip/Tuck.
- Has been featured as “Top Doctor” in US News and World Report, Pasadena Magazine, and Los Angeles Magazine.
Last modified by Dr. Andre Panossian
