Ectopic Duodenal Varices Secondary to Portal Hypertension Caused by Primary Hepatic Neuroendocrine Tumor

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.