S5877 Infiltrative Primary Hepatic Angiosarcoma: A Case Report

Introduction: Primary hepatic angiosarcoma (PHA) is a rare and aggressive malignancy associated with high mortality, accounting for 0.5%-2.0% of all primary liver cancers with a median survival rate of 1-6 months. Notably, PHA does not have a distinct clinical or radiographic presentation. Here, we present an infiltrative case of this rare disease. Case Description/Methods: We present the case of a 64-year-old-man who presented to us with confusion and ascites after a 2-month period of multiple hospitalizations. Medical history was significant for RA-ILD s/p single lung transplant more than 5 years prior, CAD, Afib, remote alcohol/tobacco use. He was first noted to have cholestatic liver enzyme elevation on outpatient laboratory work-up with Alk phos 326, aspartate aminotransferase 93, alanine aminotransferase 56, TB 1.0. Later that month he was hospitalized with general malaise, nausea, and vomiting. US showed steatosis and nonspecific GB wall thickening, no focal liver lesions or evidence of biliary obstruction. MRCP showed cirrhotic morphology and LR-3 nonspecific subcapsular parenchymal enhancement, capsular retraction in the right lobe without convincing washout, as well as new trace ascites. History was positive for heavy alcohol use 10 years prior, recent doxycycline for folliculitis and turmeric supplements for his RA, all of which were stopped. He was also on standard immunosuppression for his lung transplant. No notable environmental exposures or family history. Chronic hepatitis serologies were negative, infectious/autoimmune/metabolic/genetic etiologies were excluded, and he was discharged with presumed cryptogenic cirrhosis. He was hospitalized again with rapidly worsening ascites and underwent paracentesis. Days after discharge, he presented with grade III hepatic encephalopathy, worsening LFTs, and coagulopathy international normalized ratio 3.9 with MELD score of 28. He underwent Transjugular liver biopsy that showed spindle cell infiltration and histology confirming a diagnosis of PHA. Unfortunately, he shortly thereafter transitioned to hospice and expired within the next weeks. Discussion: This patient had nonspecific symptoms and imaging findings initially suggestive of cirrhosis. However, his rapid progression to liver failure instead suggested an insidious process leading to biopsy and diagnosis of PHA. The infiltrative form likely led to his portal hypertension and ascites. This case highlights how the variance in symptomatology and imaging makes diagnosis of PHA overall challenging, and the importance of early histopathologic diagnosis.