Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby

Kaposiform hemangioendothelioma is a vascular tumor categorized as intermediate malignancy. We experienced an autopsy of a female baby with kaposiform hemangioendothelioma with Kasabach-Merritt syndrome. She died of systemic bleeding tendency following disseminated intravascular coagulation at the age of 9 days. At autopsy, a huge main tumor, histologically kaposiform hemangioendothelioma, was discovered in the mediastinum between the right chest cavity and pericardium. Furthermore, kaposiform hemangioendothelioma with the same histology involved the lungs, heart, liver, subserosa of cardial part of the stomach, retroperitoneum around the right adrenal gland, broad ligament of the uterus, and muscular tissue around the thyroid. To date, a few previously reported cases of multifocal kaposiform hemangioendothelioma have demonstrated locally aggressive distributions mainly in bone and soft tissues. The present case with extensive distribution including visceral organs implies that kaposiform hemangioendothelioma may have higher potential to spread than considered before.