Kaposiform hemangioendothelioma: report of a case unresponsive to usual medical treatments

Abstract

Kaposiform hemangioendothelioma is an aggressive endothelial-derived spindle cell neoplasm that occurs nearly exclusively during childhood and teenage years. The lesion grows rapidly and is often associated with Kasabach-Merritt syndrome.

In this study a 24 days old male neonate who presented with an ill-defined deeply situated violaceous mass on his left arm is described. He had also anemia and life-threatening thrombocytopenia. Despite hospitalization in intensive care unit (ICU) and transfusion of platelets and packed red blood cells as well as medical managements such as oral prednisolone, intravenous (IV) methylprednisolone and interferon alpha, thrombocytopenia persisted, so surgical resection was considered. The histopathological findings were distinctive and characteristic of kaposiform hemangioendothelioma. Following surgery, the infant did not have any complications and was discharged from the hospital in good condition.

KEYWORDS: Hemangioendothelioma, Sarcoma-Kaposi, Infant-Newborn, Arm, Surgery.