Black and White Liver

Abstract

72-year-old man was admitted with fulminant heApatic failure including jaundice, coagulopathy, and encephalopathy. He described 6 months of insidious weakness and malaise. One month previously, he had been treated with doxycycline and developed progressive peripheral edema, ascites, and jaundice. He denied alcohol, high-risk behaviors, or foreign travel. His past medical history was significant for remote ocular melanoma, which was treated with radiation and monitored without recurrence. Laboratory results showed an international normalized ratio of 1.5, total bilirubin level of 17.5 mg/dL, alkaline phosphatase level of 3201 U/L, aspartate aminotransferase level of 214 U/L, and alanine aminotransferase level of 49 U/L. Physical examination showed jaundice, bilateral icterus, neurocognitive slowing, asterixis, ascites, and lower-extremity edema, without lymphadenopathy. His liver biopsy had been interpreted elsewhere as coagulative necrosis, unknown etiology, no evidence of malignancy. Out of concern for drug-induced liver failure, he was transferred to the liver transplant service for consideration of emergency transplantation. Magnetic resonance imaging showed a black and white liver pattern with abnormal restricted diffusion throughout the right hepatic lobe (axial diffusion-weighted image, 600 b-value) relative to the normal left lobe (Figure A). On additional sequences (not shown), the right hepatic lobe showed increased T2-weighted signal and decreased precontrast T1-weighted signal. Postcontrast, fat-suppressed, T1weighted images showed arterial enhancement followed by mild washout. The hepatic venous pressure gradient was consistent with portal hypertension. Transjugular liver biopsy with H&E staining (Figure B) showed near-complete replacement of the hepatic parenchyma with diffusely infiltrating malignant cells that closely mimicked the normal hepatic parenchyma. Positive immunohistochemical staining for the melanoma markers melan-A (Figure C) and HMB-45, and negative staining for S-100 (data not shown) confirmed a diagnosis of metastatic melanoma. Given the acute presentation, rapid decompensation, and degree of liver dysfunction, the patient was transitioned to comfort measures and died within days. Uveal melanoma is the most common primary ocular malignancy with an unusually high rate of metastatic disease (up to 50%), with liver being the most common site of metastases (89%). Prognosis, regardless of treatment strategy, remains poor, with a 2-year mortality rate of 90% and an approximately 1% survival rate at 5 years. Typically, the hepatic dysfunction associated with metastatic melanoma is mild. However, there have been several reports highlighting a rapidly progressive and severe form of fulminant hepatic failure that generally leads to death within days. This case highlights the particular propensity of ocular melanoma for metastasizing to the liver with