Case ReportGiant Pancreatic Myoepithelial Hamartoma in a Patient With Familial Insulin-Dependent Diabetes MellitusSharib, Jeremy MD∗; Ord, Jeffrey R. BS†; Bentley, Rex C. MD‡; Shi, Chanjuan MD, PhD‡; Blazer, Dan G. III MD§ Author Information From the ∗Department of Surgery †School of Medicine Departments of ‡Pathology §Surgery, Duke University, Durham, NC. Received for publication July 9, 2021; accepted August 29, 2022. Address correspondence to: Jeremy Sharib, MD, 2301 Erwin Rd, Duke Hospital North, HAFS 7684A, Durham, NC 27710 (e-mail: [email protected]). The authors declare no conflict of interest. Pancreas: August 2022 - Volume 51 - Issue 7 - p 830-833 doi: 10.1097/MPA.0000000000002110 Buy Metrics Abstract Pancreatic myoepithelial hamartoma is a rare, benign solid and cystic lesion of the pancreas. We present the first case of an adult with a giant myoepithelial hamartoma extending throughout the pancreas in a patient with diabetes in 4 immediate family members. The patient is a 46-year-old man presented with recurrent acute pancreatitis. Computed tomographic imaging showed that the head and body of the pancreas were replaced by a solid-cystic mass with focal calcification. Medical history includes insulin-dependent diabetes mellitus (IDDM) diagnosed at age 30. Endoscopic ultrasound-guided fine-needle aspiration showed pancreatic acinar tissue and smooth muscle without evidence of malignancy. Total pancreatectomy was performed because of the diffuse nature of the cystic disease and preexisting IDDM. The histopathologic diagnosis was consistent with myoepithelial hamartoma. In addition, there was a family history of IDDM and hamartomatous cyst resection in the paternal grandmother. We report the first case of diffuse pancreatic myoepithelial hamartoma with near total replacement of the entire pancreatic parenchyma, and the first reported case associated with a family history of heritable IDDM. Improved knowledge of the genetics, development, and malignant potential of such rare diseases is critical to determine appropriate management for patients. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.