Wadhwa S, Kim TH, Lin L, Kanel G, Saito T. Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome. World J Gastroenterol 2017; 23(13): 2443-2447 [PMID: 28428724 DOI: 10.3748/wjg.v23.i13.2443]
Corresponding Author of This Article
Takeshi Saito, MD, PhD, Department of Medicine, Molecular Microbiology and Immunology, and Pathology, Division of Gastrointestinal and Liver Diseases, Keck School of Medicine of USC, University of Southern California, 2011 Zonal Avenue, HMR 801A, Los Angeles, CA 9003, United States. saitotak@usc.edu
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastroenterol. Apr 7, 2017; 23(13): 2443-2447 Published online Apr 7, 2017. doi: 10.3748/wjg.v23.i13.2443
Hepatic angiosarcoma with clinical and histological features of Kasabach-Merritt syndrome
Sanya Wadhwa, Tae Hun Kim, Leah Lin, Gary Kanel, Takeshi Saito
Sanya Wadhwa, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA 90033, United States
Takeshi Saito, Department of Medicine, Molecular Microbiology and Immunology, and Pathology, Division of Gastrointestinal and Liver Diseases, Keck School of Medicine of USC, University of Southern California, Los Angeles, CA 90033, United States
Tae Hun Kim, Gary Kanel, Department of Pathology, University of Southern California, Keck School of Medicine, Los Angeles, CA 90033, United States
Leah Lin, Department of Radiology, University of Southern California, Keck School of Medicine, Los Angeles, CA 90033, United States
Author contributions: Wadhwa S gathered information of the case, reviewed the literature, and wrote the manuscript; Kim TH and Kanel G provided pathological interpretation of liver biopsy; Lin L provided interpretation of radiographic imaging, Saito T oversaw the manuscript preparation; all of the authors contributed to the intellectual content.
Institutional review board statement: This case report was exempt from the Institutional Review Board standards at University of Southern California.
Informed consent statement: The patient involved in this study was deceased so no informed consent was obtained.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Takeshi Saito, MD, PhD, Department of Medicine, Molecular Microbiology and Immunology, and Pathology, Division of Gastrointestinal and Liver Diseases, Keck School of Medicine of USC, University of Southern California, 2011 Zonal Avenue, HMR 801A, Los Angeles, CA 9003, United States. saitotak@usc.edu
Telephone: +1-323-4422260 Fax: +1-323-4425425
Received: October 29, 2016 Peer-review started: October 30, 2016 First decision: December 19, 2016 Revised: December 23, 2016 Accepted: January 4, 2017 Article in press: January 4, 2017 Published online: April 7, 2017 Processing time: 159 Days and 6 Hours
Abstract
Hepatic angiosarcoma is a mesenchymal tumor originating from liver sinusoidal endothelial cells. It is an extremely rare malignant neoplasm accounting for less than 1% of primary malignant liver tumors. The deregulated coagulopathy that can be seen in hepatic angiosarcoma fulfills the clinical diagnostic criteria of disseminated intravascular coagulation. However, the mechanism that governs this coagulopathy has been poorly understood. This case report provides histological evidence of the consumption of coagulation factors along with trapped platelets occurring within the tumor, which is the foundation for the concept of Kasabach-Merritt syndrome (KMS). KMS is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. However, KMS associated with angiosarcoma has not been well recognized. This case report describes, for the first time, the histological evidence of KMS that occurred in an extremely rare mesenchymal malignant tumor of the liver.
Core tip: Kasabach-Merritt syndrome (KMS) is characterized by thrombocytopenia and hyperconsumption of coagulation factors within a vascular tumor. KMS is typically seen in the pediatric population however there have been reports of KMS occurring in association with adult vascular tumors. Based on laboratory findings, it is hard to differentiate KMS from disseminated intravascular coagulation. Here, we describe, for the first time the histological evidence validating the concept of KMS.
