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.

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.