[Thoracic kaposiform hemangioendothelioma. Four consecutive cases with distinct outcome]

Kaposiform hemangioendothelioma (KHE) is a rare, frequently congenital, neoplasm associated with the Kasabach-Merritt phenomenon (KMP) and predilection for the trunk. Its clinical course is unpredictable. A child with KHE can die as a result of hemorrhage or show early spontaneous regression. In addition various and concurrent therapies can be used to treat this tumor and it is difficult to predict which treatment will be successful.

We present four consecutive cases of KHE (larger than 20 cm) of the thorax. All patients developed KMP, without skin involvement in one patient. Patient 1, a neonate with KHE occupying both hemithoraces (nearly 50% of the body surface area), died shortly after birth due to coagulopathy and generalized bleeding. Patient 2, a neonate, was treated with interferon alpha-2a and showed accelerated correction of coagulopathy and complete tumoral regression at 9 months. Patient 3 showed no response to steroids, interferon or vincristine therapy. Thrombocytopenia (platelet count 40,000) persisted for 8 years and was resolved by administration of aspirin plus ticlopidine, without tumoral disappearance. Patient 4 underwent incomplete removal of the tumor on her right chest wall after showing no response to antiangiogenic therapy. The coagulopathy persisted and a second radical surgical procedure resolved KMP.

Given the variable response to pharmacological treatment and the scant possibilities of surgical resection, the management of KHE with KMP must include a multidisciplinary approach. As little is known about the pathogenesis of these highly aggressive vascular tumors, further molecular research is needed to understand their long-term behavior.