Multifocal Kaposiform Hemangioendothelioma Causing Massive Fetal Chylous Ascites

Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in soft tissues of the extremity and rarely in the retroperitoneum. We report a unique case of isolated massive fetal ascites attributed to KHE, involving the retroperitoneum and multiple visceral organs...

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Bibliographic Details
Published in:Fetal and pediatric pathology 2017-04, Vol.36 (2), p.162-167
Main Authors: An, Hyo Jung, Jung, Eun Jung, Kang, Mi Seon, Cho, Hwa Jin, Nam, Kyung Han, Byun, Jung Mi, Jeong, Dae Hoon, Kim, Young Nam
Format: Article
Language:English
Subjects:
Adult
Chylous Ascites - diagnosis
Chylous Ascites - etiology
Chylous Ascites - pathology
Fatal Outcome
Female
Fetal Diseases - diagnosis
Fetal Diseases - pathology
Fetus - pathology
Hemangioendothelioma - complications
Hemangioendothelioma - diagnosis
Hemangioendothelioma - pathology
Humans
Kasabach-Merritt Syndrome - complications
Kasabach-Merritt Syndrome - diagnosis
Kasabach-Merritt Syndrome - pathology
Pregnancy
Sarcoma, Kaposi - complications
Sarcoma, Kaposi - diagnosis
Sarcoma, Kaposi - pathology
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Summary:Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that usually occurs in soft tissues of the extremity and rarely in the retroperitoneum. We report a unique case of isolated massive fetal ascites attributed to KHE, involving the retroperitoneum and multiple visceral organs, along with the Kasabach-Merritt phenomenon. We suspect that retroperitoneal KHE might have caused massive fetal ascites because of its high potential to invade the lymphatic vessels aggressively in the retroperitoneal space, which possibly permits intestinal lymph leakage into the peritoneal cavities.
ISSN:1551-3815
1551-3823
DOI:10.1080/15513815.2016.1261973