Peribronchial Arteriovenous Malformation with Cowden Syndrome: A Rare Case Report

Cowden syndrome (CS) is a rare hereditary disorder caused by a germline variant of the phosphatase and tensin homolog, associated with multiple hamartomatous lesions occurring in various organs. Additionally, although rare, arteriovenous malformations (AVMs) with CS are found in the skin, brain, and spinal cord; however, peribronchial AVMs have not been previously reported. Herein, we report a rare case of a peribronchial AVM in a 30-year-old man with CS who presented with hemoptysis. Computed tomography (CT) revealed an AVM around the left upper bronchus, which was mainly fed by the left bronchial artery and drained into the left inferior pulmonary vein. Under video-assisted thoracic surgery, ligation of the feeding and draining vessels was performed. The AVM remarkably decreased in size one month after the surgery. This case highlights the need for whole-body contrast-enhanced CT to screen for AVMs and the importance of identifying feeding and draining vessels for optimal treatment methods.