Sinusoidal Hepatic Angiosarcoma on a Background of Non-cirrhotic Portal Hypertension and Nodular Regenerative Hyperplasi
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CASE REPORT
Sinusoidal Hepatic Angiosarcoma on a Background of Non-cirrhotic Portal Hypertension and Nodular Regenerative Hyperplasia Masquerading as Acute on Chronic Liver Failure Mukul Vij 1 & Vaibhav Patil 2 & Abdul Hakeem 2 & Mettu Srinivas Reddy 2 & Ilankumaran Kaliamoorthy 3 & Mohamed Rela 2,4
# Springer Science+Business Media, LLC, part of Springer Nature 2020
Abbreviations CT Computed tomography ACLF Acute on chronic liver failure NRH Nodular regenerative hyperplasia HA Hepatic angiosarcoma NCPH Non-cirrhotic portal hypertension
Introduction Hepatic angiosarcomas (HAs) are rare malignant tumors with variable clinical and radiologic characteristics. In almost all cases, HA pathologic confirmation is required for definite diagnosis. Four major histologic patterns of HA were described recently. These include non-mass forming and sinusoidal growth, non-mass forming and peliotic growth, mass forming and vasoformative, and mass forming and epithelioid or spindled [1]. Non-cirrhotic portal hypertension (NCPH) is a vascular liver disease of unknown etiology characterized by features of portal hypertension in the absence of significant fibrosis [2]. Nodular regenerative hyperplasia (NRH) represents an adaptive hyperplastic reaction of hepatocytes to mechanical or functional abnormalities of hepatic microvasculature due to imbalances between arterial and portal venous
blood flow and is characterized by multiple regenerative nodules distributed throughout the liver parenchyma without a fibrous rim [3]. Herein, we describe the first case of HA with sinusoidal growth pattern discovered incidentally in an explant liver having NCPH and NRH. The patient presented with acute on chronic liver failure (ACLF) and underwent living donor living transplantation at our institution.
Case Report The present case concerns a 57-year-old gentleman diagnosed with chronic liver disease 1 year ago, rapidly deteriorated with refractory ascites, jaundice, and one episode of grade II hepatic encephalopathy. Triphasic computed tomography (CT) abdomen showed features of cirrhosis, prominent portosystemic collaterals, moderate ascites, patent portal and hepatic venous vasculature, and no mass forming lesion (Fig. 1a–c). The upper gastrointestinal endoscopy showed low-risk esophageal varices with mild portal hypertensive gastropathy. Etiological workup was negative except for marginally elevated autoantibody levels (antinuclear antibody and anti-smooth muscle antibody). A diagnosis of ACLF with probable autoimmune etiology was considered. Glucocorticoid therapy was not initiated due to recent sepsis.
* Mukul Vij [email protected] Vaibhav Patil [email protected] Abdul Hakeem [email protected] Mettu Srinivas Reddy [email protected] Ilankumaran Kaliamoorthy [email protected]
Mohamed Rela [email protected] 1
Department of Pathology, Dr Rela Institute & Medical Center, Bharat Institute of Higher Education and Research, Chennai, India
2
Institute of Liver Disease & Transplantation, Dr Rela Instit
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