Kaposi-like infantile hemangioendothelioma. A distinctive vascular neoplasm of the retroperitoneum.
Retroperitoneal soft-tissue tumors are rare in infancy. In this report, we describe a distinctive hemangioendothelioma occurring in the retroperitoneum of a 10-month-old baby girl. This lesion was complicated by obstructive jaundice, intestinal obstruction, and thrombocytopenia (Kasabach-Merritt syndrome) leading to intracranial hemorrhage. The microscopic features of this tumor, characterized by infiltrative lobules of spindle cells and capillaries, are distinct from those of other well-recognized vasoformative tumors. In some areas, the tumor shows a striking resemblance to Kaposi's sarcoma; criss-crossing fascicles of spindle cells are interspersed with narrow vascular spaces, but PAS-positive hyaline globules are absent. The tumor can also be distinguished from the cellular hemangioma of childhood by its well-formed spindle cell fascicles. Several histologically similar cases have been reported. All of them occurred in the retroperitoneum of infants and were frequently complicated by Kasabach-Merritt syndrome. We therefore propose the designation "Kaposi-like infantile hemangioendothelioma" for this unusual neoplasm.
Tsang
W Y
WY
Institute of Pathology, Queen Elizabeth Hospital, Hong Kong, Kowloon.
Chan
J K
JK
D001344:Autopsy; D003937:Diagnosis, Differential; D005260:Female; D006390:Hemangioendothelioma / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D012514:Sarcoma, Kaposi / Q000175:diagnosis / Q000378:metabolism / Q000473:pathology*; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor