Hospital of the University of Pennsylvania Philadelphia, PA, United States
Sara E. Chapin, MD1, Jacob Sweeney, MD1, Emma Furth, MD1, Zhaohai Yang, MD, PhD2, Kenneth Rothstein, MD1 1Hospital of the University of Pennsylvania, Philadelphia, PA; 2University of Pennsylvania Health System, Philadelphia, PA
Introduction: Hepatic Angiosarcoma, a rare neoplasm of endothelial origin, accounts for 2% of primary liver malignancies and conveys a poor prognosis. Here, we describe a case of liver failure in a liver transplant recipient 13 years post-transplant caused by Primary Hepatic Angiosarcoma.
Case Description/Methods: A 62-year-old-man with history of cirrhosis due to Hepatitis C requiring liver transplantation thirteen years prior presented to hepatology clinic with pruritis, right upper quadrant abdominal pain and jaundice. Chronic rejection was suspected, however, liver biopsy revealed only a venous outflow pattern of injury. Magnetic Resonance Angiography of the liver showed cirrhosis and portal hypertension with diffuse regenerative nodules, fibrosis, and biliary duct dilation. Due to concern for malignancy, biopsy of one nodule was performed and demonstrated sinusoidal dilation, organizing thrombosis, parenchymal loss, and fibrosis without evidence of malignancy. One month later, the patient was hospitalized for decompensated liver failure and underwent liver transplantation. Pathology from the explanted liver exhibited multiple, small foci with irregular vascular channels lined by atypical cells. Immunohistochemistry revealed ERG and CD31 positive tumor cells. Ki-67 proliferative index was elevated to 25%, and P53 was diffusely positive. The morphology and immunoprofile supported a diagnosis of Angiosarcoma.
Discussion: Hepatic Angiosarcoma is a challenging diagnosis due to its vague clinical presentation, variable radiographic findings, and lack of specific tumor markers. In this case, two liver biopsies revealed findings of venous outflow obstruction without diagnostic features of malignancy. A similar venous outflow obstruction pattern was observed in the explant liver, with features of angiosarcoma only identified in small foci. Challenges posed to pathologists in cases of Hepatic Angiosarcoma are at least two-fold. First, well-differentiated Angiosarcoma is often hypocellular with subtle nuclear changes impeding diagnosis of malignancy. Second, the sinusoidal-type shares similar microscopic features to venous outflow obstruction, and the true diagnostic area may not be biopsied.Liver transplantation for Angiosarcoma is contraindicated due to high recurrence rates and poor post-transplant survival. In cases of liver failure suspicious for malignancy, open liver biopsy involving multiple sites should be considered prior to liver transplantation.
Figure: A. Magnetic Resonance Angiography (MRA) of the liver demonstrates a cirrhotic morphology with several round T2 hyperintense structures throughout the liver. B. On delayed MRA imaging, the liver largely fills in with patchy areas of nonenhancement. No discrete suspicious lesion identified. C. Biopsy sample with centrizonal sinusoidal dilation as well as centrizonal perisinusoidal fibrosis (H&E stain). D: Explant with Angiosarcoma. E: Ki-67 immunostain shows elevated proliferation index in tumor. F: P53 staining shows diffuse positive staining in tumor cells compatible with mutant phenotype.
Disclosures:
Sara Chapin indicated no relevant financial relationships.
Jacob Sweeney indicated no relevant financial relationships.
Emma Furth indicated no relevant financial relationships.
Zhaohai Yang indicated no relevant financial relationships.
Kenneth Rothstein indicated no relevant financial relationships.
Sara E. Chapin, MD1, Jacob Sweeney, MD1, Emma Furth, MD1, Zhaohai Yang, MD, PhD2, Kenneth Rothstein, MD1. P1841 - Hepatic Angiosarcoma as a Late Cause of Liver Failure in a Liver Transplant Recipient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.
