Metastatic Neuroendocrine Tumor of the Pancreas Presenting With Gastric Variceal Hemorrhage

Abstract

Pancreatic neuroendocrine tumors (PNETs) are rare, typically indolent neoplasms with a reported incidence of 0.43 per 100,000 persons per year. PNETs can occasionally invade adjacent vasculature structures with resultant vessel occlusion. This differs from other pancreatic malignancies which typically induce venous thrombosis. Gastric variceal hemorrhage is a rare complication of PNETs which can occur from tumor invasion of the splenic vein. A 56 year-old woman with a past medical history of pancreatic neuroendocrine tumor metastatic to the liver presented with a 2 week history of nausea and a 1 day history of hematemesis. She was tachycardic but normotensive on admission. Serum hemoglobin had decreased from 14.4 g/dL a week prior to 10.6 g/dL on admission. Emergent esophagogastroduodenoscopy in the critical care unit demonstrated a hematocystic spot on a gastric varix with additional fundal and gastric varices. No esophageal varices were seen. Computerized tomography scan of the abdomen demonstrated vascular occlusion of the splenic vein at the hilum from local invasion of the splenic vein by a known pancreatic tail neuroendocrine tumor. There were unchanged liver metastatic lesions without radiologic evidence of cirrhosis. Notably, liver enzymes and serum lipase were normal. The patient was transfused with 1 unit of packed red blood cells and had no further bleeding after selective embolization of the gastric varices by the interventional radiology service. Splenic vein thrombosis (SVT) and gastric variceal hemorrhage are known complications of pancreatitis and pancreatic adenocarcinoma. Venous thrombosis occurs from local inflammation, fibrotic tendencies, and possibly a hypercoagulable state. Splenic vein occlusion is a rare complication of PNETs occurring from local tumor invasion of the splenic vein. SVT and occlusion can cause regional portal hypertension and upper gastrointestinal bleeding (UGIB). Splenectomy and possibly local resection are the standard therapies for PNETs with associated gastric variceal hemorrhage. Radiologic gastric variceal embolization is a treatment option which can be particularly useful in patients with known metastatic disease. Endoscopic injection with cyanoacrylate glue is an additional therapeutic modality but is not Food and Drug Administration approved in the United States. SVT and gastric variceal hemorrhage are rare complications that should be included in the differential diagnosis of UGIB in patients with PNETs.2006_A Figure 1. Gastric varices with a hematocystic spot on a varix2006_B Figure 2. Yellow arrow: Hepatic Metastasis Red arrow: Pancreatic neuroendocrine mass with apparent splenic vein thrombosis