Kaposiform hemangioendothelioma associated with nonimmune fetal hydrops.
We describe the case of a 31-week fetus who died in utero with an invasive retroperitoneal kaposiform hemangioendothelioma. This rare vascular neoplasm usually presents as a localized violaceous skin lesion in infants and behaves in a benign fashion; however, kaposiform hemangioendothelioma may present as an invasive neoplasm of the chest or abdominal cavity, where it can lead to the Kasabach-Merritt syndrome, which consists of thrombocytopenia, consumptive coagulopathy, and microangiopathic anemia in association with a vascular anomaly. The case we describe is unique in that the tumor presented in utero and led to intrauterine nonimmune fetal hydrops. Kaposiform hemangioendothelioma has been described in utero; however, to our knowledge, intrauterine fetal death as a direct consequence has not been reported previously in the literature.
Martinez
Antonio E
AE
Arkadi M. Rywlin MD Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, Fla 33140, USA. amartin2@msmc.com
Robinson
Morton J
MJ
Alexis
John B
JB
D005260:Female; D005313:Fetal Death / Q000209:etiology; D005315:Fetal Diseases / Q000175:diagnosis / Q000473:pathology*; D006390:Hemangioendothelioma / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology*; D009361:Neoplasm Invasiveness; D011247:Pregnancy; D012186:Retroperitoneal Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology*