title: Multifocal kaposiform hemangioendothelioma in multiple visceral organs: an autopsy of 9-day-old female baby.
abstract: 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.
authors.affiliation: Department of Pathology, Graduate School of Medicine, University of Tokyo, Tokyo 113-0033, Japan; Department of Molecular Pathology, Tokyo Medical University, Tokyo 160-8402, Japan. Electronic address: nakaya@ks.kyorin-u.ac.jp.
authors.forename: Takeo
authors.initials: T
authors.lastname: Nakaya
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo 113-0033, Japan.
authors.forename: Kiyoko
authors.initials: K
authors.lastname: Morita
authors.affiliation: Department of Molecular Pathology, Tokyo Medical University, Tokyo 160-8402, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kurata
authors.affiliation: Department of Pathology, Graduate School of Medicine, University of Tokyo, Tokyo 113-0033, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Ushiku
authors.affiliation: Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo 113-0033, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Igarashi
authors.affiliation: Department of Molecular Pathology, Tokyo Medical University, Tokyo 160-8402, Japan.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Kuroda
authors.affiliation: Department of Pathology, Graduate School of Medicine, University of Tokyo, Tokyo 113-0033, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Fukayama
mesh_terms: D005260:Female; D006390:Hemangioendothelioma / Q000150:complications / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D059885:Kasabach-Merritt Syndrome / Q000150:complications / Q000473:pathology*; D012514:Sarcoma, Kaposi / Q000150:complications / Q000473:pathology*
keywords: Intermediate malignancy; Kaposiform hemangioendothelioma; Kasabach-Merritt syndrome; Multifocal organ involvement; Vascular tumor