Kasabach-Merrit syndrome and adult hepatic epithelioid hemangioendothelioma an unusual association

Journal of Hepatology
Bernardo FriderRaquel Bistoletti

Abstract

Kasabach-Merrit syndrome (KMS) is very rarely observed in adults associated with visceral hemangiomas. Hepatic epithelioid hemangioendothelioma (HEHE) is a very rare clinical entity with an intermediate malignant potential and a mortality rate of 20-30%. We described a case of KMS associated with HEHE in a 70 year old male patient who presented coagulation abnormalities, low platelet count and high fibrin degradation products that were typical features of KMS. Histopathology of the hepatic tissue and a positive immunostaining for endothelial markers confirmed the diagnosis of an HEHE. The first description of KMS was made in children in 1940. It was described as a "consumptive coagulopathy with capillary hemangiomas". However, recent reports have shown that this condition could be associated with kaposiform hemangioendotheliomas, an aggressive form of giant hemangioma. A giant hemangioma in adults as well as an hemangioendothelioma in children could be associated KMS, but an HEHE has been rarely reported in association with this syndrome in adult patients.

References

Jan 1, 1984·Virchows Archiv. A, Pathological Anatomy and Histopathology·M FukayamaM Koike
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Citations

May 17, 2007·The American Journal of the Medical Sciences·Fares SalamehItay Maza
Dec 7, 2013·HPB : the Official Journal of the International Hepato Pancreato Biliary Association·Ryan M ThomasEddie K Abdalla
Feb 5, 2015·Gastroenterology Research and Practice·Sara MassironiDario Conte
Mar 22, 2014·Molecular and Clinical Oncology·Kaoru NagaoTomoatsu Kimura
Dec 12, 2018·Bulletin du cancer·Sophie CousinNicolas Penel
May 18, 2021·World Journal of Surgery·Mohamad A ChahrourFaek R Jamali

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