Intraductal papillary mucinous neoplasm of the pancreas (IPMN): Pathophysiology and clinical manifestations
- Sunil G Sheth, MD
Sunil G Sheth, MD
- Assistant Professor of Medicine
- Harvard Medical School
- Douglas A Howell, MD, FASGE, FACG
Douglas A Howell, MD, FASGE, FACG
- Section Editor — EUS/ERCP
- Assistant Clinical Professor of Medicine, Tufts Medical School Director,
- Pancreaticobiliary Center Director, Advanced Interventional Endoscopy Fellowship, Maine Medical Center
- Tara S Kent, MD, FACS
Tara S Kent, MD, FACS
- Assistant Professor of Surgery
- Harvard Medical School
- Beth Israel Deaconess Medical Center
Cystic neoplasms of the pancreas include serous cystic tumors, mucinous cystic neoplasms, solid pseudopapillary neoplasms, cystic islet cell tumors, and intraductal papillary mucinous neoplasms of the pancreas (IPMNs) [1-3]. IPMNs have also been referred to as mucinous duct ectasias and intraductal papillary mucinous tumors. IPMNs are potentially malignant intraductal epithelial neoplasms that are grossly visible (>1 cm) and are composed of mucin-producing columnar cells. The lesions show papillary proliferation, cyst formation, and varying degrees of cellular atypia [4,5]. IPMNs may involve the main pancreatic duct, the branch ducts, or both. (See "Classification of pancreatic cysts".)
This topic will review the pathophysiology and clinical manifestations of IPMNs. The diagnosis and treatment of IPMNs, as well as an overview of pancreatic cystic neoplasms, are presented separately. (See "Intraductal papillary mucinous neoplasm of the pancreas (IPMN): Evaluation and management" and "Classification of pancreatic cysts" and "Pancreatic cystic neoplasms: Clinical manifestations, diagnosis, and management".)
Intraductal papillary mucinous neoplasm of the pancreas (IPMN) was first described in 1982 when four patients with pancreatic carcinoma and favorable outcomes were reported. The patients were noted to have dilated main pancreatic ducts, patulous ampullary orifices, and mucus secretion from the pancreatic duct . With time, the incidence of IPMN has increased, largely due to increased diagnosis . Prior to 1999, the distinction between IPMN and mucinous cystic neoplasm had not been clarified, so many lesions previously classified as MCNs may have, in fact, been IPMNs . In addition, improvements in imaging technology have led to more accurate identification of cystic pancreatic lesions.
The true incidence of IPMN is not known because many IPMNs are small and asymptomatic. A series of 2832 consecutive computed tomography scans performed in adults without a history of pancreatic lesions or factors predisposing to pancreatic disease found pancreatic cysts in 73 (2.6 percent) . In a similar study of 616 consecutive patients undergoing magnetic resonance imaging, the incidence of pancreatic cysts was higher (13.5 percent), with a median diameter of 6 mm . Many of these were likely IPMNs since it is thought that IPMNs account for 1 to 3 percent of exocrine pancreatic neoplasms and 20 to 50 percent of cystic pancreatic neoplasms [11-13].
The male-to-female ratio for main duct IPMN has varied in reports from 1.1 to 3:1, and for branch duct IPMN it has varied from 0.7 to 1.8:1 . The ratio varies geographically, with a male predominance in Japan and Korea and a more even distribution or female predominance in the United States and Europe. The typical age at presentation is in the fifth to seventh decade  (table 1).
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