An Autopsy Case of Myotonic Dystrophy Type 1 With Pancreatic Intraductal Papillary Mucinous Neoplasm

Here, we present an autopsy case of long-standing myotonic dystrophy type 1 (DM1) in a patient who developed a pancreatic intraductal papillary mucinous neoplasm (IPMN). DM1 is a progressive genetic disorder that affects multiple organs, including the respiratory muscles. Several nationwide registry-based cohort studies have suggested that patients with DM1 have an increased risk of developing pancreatic cancers such as pancreatic ductal adenocarcinoma (PDAC). Pancreatic IPMNs are thought to progress from benign neoplasms to invasive cancers, and surgical specimens are usually required for the pathological diagnosis of pancreatic IPMNs. Although certain risk factors for developing pancreatic IPMNs reportedly overlap with those for PDAC, few cases of DM1 with pancreatic IPMNs have been reported. This is partly because pancreatectomy is associated with relatively high morbidity and mortality rates and few patients with DM1 who are suspected of having pancreatic IPMNs are candidates for surgical resection. Therefore, cases of DM1 with histopathologically diagnosed pancreatic IPMNs are rare, and the accumulation of such cases is important for understanding the association between DM1 and pancreatic IPMNs.