A Rare Cause of Fulminant Liver Failure

Abstract

Primary hepatic angiosarcoma (HAS) is a rare cause of hepatic malignancy with an annual incidence of 200 cases worldwide. HAS is a high grade vascular neoplasm and is often caused by exposure to toxins such as vinyl chloride, anabolic steroids, arsenic, and radiation. Most patients are men over the age of 60 who generally present with abdominal pain, ascites, and hepatomegaly. It is extremely rare for patients with HAS to present with fulminant hepatic failure. An 81 year old male presented to an outside hospital with one week of confusion and slurred speech. Initial evaluation included a CT of the head that was unremarkable, a complete blood count notable for leukocytosis, and urinalysis concerning for infection. He was treated for sepsis secondary to a urinary tract infection but continued to worsen and was transferred to our tertiary care facility. Upon transfer, laboratory analysis showed a white blood cell count of 49,800 /uL with neutrophil predominance, platelet count of 82000/ uL, international normalized ratio of 17, aspartate aminotransferase of 204 unit/L, alanine aminotransferase of 91 unit/L, alkaline phosphatase of 321 unit/L, total bilirubin of 2.4mg/dL, and creatinine of 2.21mg/dL. Peripheral smear was negative for schistocytes and blast cells. Haptoglobin and fibrinogen levels were within normal limits. Infectious work up, including blood and urine cultures and viral serologies, was negative throughout his stay. His lactic acid was elevated on transfer at 7.2mmol/L and continued to rise daily. A PET scan revealed diffuse liver uptake concerning for non-specific hepatitis, although all viral and autoimmune panels were negative. The patient's mental status deteriorated and he developed circulatory shock with oliguric renal failure requiring transfer to the ICU for intubation, vasopressor support, and continuous veno-venous hemofiltration. He had multiple GI bleeds during this time requiring transfusion of blood products. A liver biopsy was performed that showed invasive angiosarcoma. At this point, given the poor prognosis of multiple organ failure combined with a treatment resistant malignancy, the patient's family elected to transition to a comfort plan of care. The patient expired within hours. While HAS is the most common sarcoma arising in the liver, it is an uncommon primary hepatic malignancy and rarely presents with fulminant hepatic failure.