Diagnosis and treatment of intraductal papillary mucinous neoplasm of the pancreas
- 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
Intraductal papillary mucinous neoplasms of the pancreas (IPMNs) are potentially malignant intraductal epithelial neoplasms that are grossly visible (>10 mm) and are composed of mucin-producing columnar cells. The lesions show papillary proliferation, cyst formation, and varying degrees of cellular atypia [1,2].
IPMNs may involve the main pancreatic duct, the branch ducts, or both. Whereas patients with branch duct lesions are at lower risk for developing malignancy (approximately 20 percent at 10 years), patients with IPMN involving the main duct are at high risk (approximately 70 percent). As a result, these lesions need to be accurately diagnosed and characterized so that appropriate treatment can be recommended. (See "Pathophysiology and clinical manifestations of intraductal papillary mucinous neoplasm of the pancreas", section on 'Classification' and "Pathophysiology and clinical manifestations of intraductal papillary mucinous neoplasm of the pancreas", section on 'Pancreatic malignancy'.)
This topic will review the diagnosis and treatment of IPMNs. The pathophysiology and clinical manifestations of IPMNs and an overview of pancreatic cystic neoplasms are discussed separately. (See "Pathophysiology and clinical manifestations of intraductal papillary mucinous neoplasm of the pancreas" and "Classification of pancreatic cysts" and "Pancreatic cystic neoplasms".)
The differential diagnosis of intraductal papillary mucinous neoplasm of the pancreas (IPMN) includes chronic obstructive pancreatitis, mucinous cystic tumors of the pancreas, and, rarely, pancreatic ductal adenocarcinoma. Our approach is to attempt to confirm a diagnosis in patients with cysts ≥10 mm in largest diameter because identification of the type of cyst will guide treatment recommendations (eg, surgical removal is suggested for mucinous cystic neoplasms in good surgical candidates). In addition, we evaluate all cysts that have concerning features on imaging (eg, mural nodularity, dilated pancreatic duct). (See "Classification of pancreatic cysts" and "Pancreatic cystic neoplasms", section on 'Approach to evaluation'.)
Our approach differs from that of a 2015 guideline from the American Gastroenterological Association (AGA), which does not recommend additional evaluation if the lesion is <30 mm in size, there is no pancreatic ductal dilation, and there are no solid components within the cyst . Instead, the AGA guideline recommends obtaining magnetic resonance imaging (MRI) in one year and then every two years for a total of five years, provided there is no change in cyst size or characteristics. After five years, it is recommended that surveillance be discontinued if there is no change. The review notes, however, that there is insufficient evidence to make definitive recommendations, and the approach may need to be individualized on a case by case basis.
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- Endoscopic evaluation
- - Endoscopic retrograde cholangiopancreatography
- - Endoscopic ultrasound
- - Pancreatoscopy
- - Intraductal ultrasound
- Radiographic evaluation
- - CT scan and magnetic resonance imaging
- - Positron emission tomography
- Serum tumor markers
- Role of adjuvant therapy for invasive cancer
- PROGNOSIS AND SURVEILLANCE
- Following patients who do not undergo surgery
- Follow-up after surgery
- Surveillance for extrapancreatic malignancies
- SUMMARY AND RECOMMENDATIONS