Abstract / Summary
ABSTRACT Primary hepatic neuroendocrine tumors (PHNETs) are rare, often presenting with abdominal pain, carcinoid syndrome, or incidental imaging findings. PHNET-associated portal hypertension is uncommon. A 45-year-old man with known PHNET presented with massive upper gastrointestinal bleeding from a duodenal ectopic varix and hemorrhagic shock. Emergent esophagogastroduodenoscopy achieved hemostasis with hemoclips. He subsequently developed high-serum-ascites albumin gradient (1.4 g/dL); imaging showed a 23-cm hepatic mass, portal vein dilatation, and splenomegaly. Liver biopsy confirmed a well-differentiated neuroendocrine neoplasm without cirrhosis, supporting tumor-related, noncirrhotic portal hypertension likely from mechanical vascular compression. This case highlights the rare cause of variceal bleeding due to noncirrhotic portal hypertension in patients with PHNET.