A Unicorn Disease: The Large Duct Variant of Invasive Ductal Adenocarcinoma of the Pancreas

Large duct adenocarcinoma (LDA) is a rare histopathological variant of pancreatic ductal adenocarcinoma (PDAC) that closely mimics intraductal papillary mucinous neoplasm (IPMN). We present a 74-year-old female diagnosed with LDA in 2017. She was initially managed with chemotherapy and laparoscopic distal pancreatectomy. After five years of stable disease on systemic chemotherapy, she was referred to us to explore further definitive treatments. We used a multidisciplinary approach with curative-intent cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC), followed by oral maintenance chemotherapy. Subsequent scans showed stable disease; she eventually underwent neoadjuvant radiation and surgery with intraoperative radiation therapy (IORT) and achieved remission.