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Fatal acute liver failure in a child with metastatic gastric adenocarcinoma.

A 17-year-old white male presented with a 2 month history of burning epigastric pain initially relieved by proton pump inhibitors. Anorexia, fatigue, and recurring low-grade fever began 1 month before presentation. For several days before presentation, he experienced repeated episodes of nonbilious, nonbloody emesis. He had lost 10 pounds in 2 months. He had no previous history of abdominal pain or gastroesophageal reflux. The maternal grandfather had had a brain tumor, and the paternal grandmother had had breast cancer. On physical examination, the patient was afebrile. He was pale. He was in no respiratory distress. There were enlarged lymph nodes in the right supraclavicular, right submandibular, and bilateral inguinal areas. The right hepatic lobe was enlarged, projecting 10 cm below the right costal margin. The spleen was not enlarged. Laboratory test results included an aspartate aminotransferase (AST) of 282 (normal 10Y41) U/L, alanine transaminase (ALT) 138 (normal 24Y54) U/L, total bilirubin 0.6 (normal G 0.8) mg/dL, prothrombin time 17.3 (normal 10.3Y12.3) seconds, International Normalized Ratio (INR) 2.45 (normal 0.8Y1.4), and partial thromboplastin time 51.9 (normal 24.5Y40.3) seconds. Alpha-fetoprotein was 2.6 (normal 0.6Y3.9) ng/mL, lactic dehydrogenase 555 (normal 117Y217) U/L, and carcinoma embryonic antigen 96.9 (normal 0Y2.4) ng/mL. Hemoglobin was 9.0 (normal 11Y14.5) g/dL, white cells 10,100/KL (normal 3.84Y9.84), and platelets 83,000/KL (normal 175Y330). Blood urea nitrogen and creatinine were normal. Serologic testing for hepatitis A, B, C, and E viruses and HIV was negative. Polymerase chain reaction testing for cytomegalovirus and Epstein-Barr virus was negative. Within 48 hours of admission, ALT rose to 693 U/L, AST to 1727 U/L, bilirubin to 4.8 mg/dL (conjugated 3.2), and prothrombin time to 19.4 seconds (INR 3.3). Computerized tomography showed a large liver with near-complete replacement of parenchyma by nodular masses. There was minimal ascites (Fig. 1). Transjugular liver biopsy demonstrated poorly differentiated adenocarcinoma with widespread necrosis. Subsequently, the patient experienced hematemesis. Upper endoscopy revealed a small, ulcerated, and hemorrhagic mass on the greater curve of the gastric antrum with partial gastric outlet obstruction (Fig. 2). Grasp biopsies of the mass demonstrated ulcerated gastric epithelium without Helicobacter pylori. Despite supportive care that included transfusions of packed red cells, platelets, and fresh frozen plasma, hepatic function deteriorated in the next 96 hours with mental status changes consistent with hepatic encephalopathy (plasma ammonia 254 Kg/dL; normal 29Y54). After respiratory failure requiring mechanical ventilation and anuria developed, support was withdrawn in consultation with the patient’s family. Autopsy confirmed that the antral mass contained sheets of malignant cells with rare intracytoplasmic vacuoles (signet ring cells) identical with those seen in the liver. The cells invaded the submucosa and muscularis propria (Figs. 3 and 4). Local tumor extension was present without evidence of primary tumor elsewhere.