Pancreatic arteriovenous malformation (P-AVM) is an extremely rare vascular anomaly characterized by abnormal connections between arteries and veins bypassing the capillary network. Less than 200 cases have been reported worldwide, and standardized treatment guidelines have not yet been established. A 72-year-old man presented with abdominal distension, diarrhea, and appetite loss. Contrast-enhanced abdominal computed tomography revealed a 5 cm mass in the pancreatic tail with multiple feeding and draining vessels, a portosystemic shunt, and moderate ascites, suggesting a P-AVM. Endoscopic ultrasonography revealed a hypoechoic area in the pancreatic tail measuring 50 mm in diameter. Esophagogastroduodenoscopy revealed F2-type esophageal varices. Based on imaging findings and clinical history, the patient was diagnosed with P-AVM, and Osler-Weber-Rendu disease was excluded. Initial treatment with transarterial embolization was attempted, but proved insufficient due to high blood flow and multiple feeders. Surgical resection via distal pancreatectomy was subsequently performed to alleviate portal hypertension and resolve abdominal symptoms, although significant intraoperative bleeding occurred. This case highlights the diagnostic and therapeutic challenges of P-AVMs, particularly in high-flow lesions with complex vascular anatomy. Surgical resection remains the definitive treatment for symptomatic P-AVM and effectively resolves the associated complications. The insights gained from this case may contribute to the clinical management of this rare condition.

Various treatment methods are available for the treatment of pancreatic arteriovenous malformation (P-AVM); however, there are no established treatment options for asymptomatic P-AVM.

A 47-year-old and a 50-year-old male patients sought treatment for P-AVM in the pancreas, which was incidentally detected during routine abdominal computed tomography and magnetic resonance imaging conducted as part of a health check-up. They underwent transcatheter arterial embolization (TAE), and over the course of a 9-year follow-up period, the AVM did not worsen and was asymptomatic.

TAE can be considered as an alternative treatment option for P-AVM in selective cases where patients are asymptomatic or have a high surgical risk.

Pancreatic arteriovenous malformation is a rare entity (0.9%). There are about 200 articles available in PubMed. This review article includes 86 published articles, with 117 cases published after 2000. The median age at diagnosis was 51, and most of the patients were male (87.0%). The symptoms included pain, bleeding, pancreatitis, ulcers in the duodenum or stomach, varix formation, jaundice, and ascites. The diagnostic modalities were angiography, contrast-enhanced CT, MRI, and/or Ultra Sound. The most common treatments were surgery and embolization. The clinical success rate of embolization reported was 57.7%. The tailored embolization based on each agio-architecture had a clinical success rate of 80%. If embolic therapy is ineffective, surgical intervention should be considered.