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Review
. 2010;15(12):1294-309.
doi: 10.1634/theoncologist.2010-0151. Epub 2010 Dec 8.

Intraductal papillary mucinous tumors of the pancreas: biology, diagnosis, and treatment

Robert Grützmann  1 , Marco Niedergethmann, Christian Pilarsky, Günter Klöppel, Hans D Saeger
Affiliations
Review

Intraductal papillary mucinous tumors of the pancreas: biology, diagnosis, and treatment

Robert Grützmann et al. Oncologist. 2010.

Abstract

Pancreatic intraductal papillary mucinous neoplasms (IPMNs) rank among the most common cystic tumors of the pancreas. For a long time they were misdiagnosed as mucinous cystadenocarcinoma, ductal adenocarcinoma in situ, or chronic pancreatitis. Only in recent years have IPMNs been fully recognized as clinical and pathological entities, although their origin and molecular pathogenesis remain poorly understood. IPMNs are precursors of invasive carcinomas. When resected in a preinvasive state patient prognosis is excellent, and even when they are already invasive, patient prognosis is more favorable than with ductal adenocarcinomas. Subdivision into macroscopic and microscopic subtypes facilitates further patient risk stratification and directly impacts treatment. There are main duct and branch duct IPMNs, with the main duct type including the intestinal, pancreatobiliary, and oncocytic types and the branch duct type solely harboring the gastric type. Whereas main duct IPMNs have a high risk for malignant progression, demanding their resection, branch duct IPMNs have a much lower risk for harboring malignancy. Patients with small branch duct/gastric-type IPMNs (<2 cm) without symptoms or mural nodules can be managed by periodic surveillance.

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Conflict of interest statement

Disclosures: Robert Grützmann: None; Marco Niedergethmann: None; Christian Pilarsky: None; Günter Klöppel: None; Hans D. Saeger: None.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors or independent peer reviewers.

Figures

Figure 1.
Figure 1.
Types and biology of IPMN. Abbreviations: BD-IPMN, branch duct IPMN; IPMN, intraductal papillary mucinous neoplasm; MD-IPMN, main duct IPMN.
Figure 2.
Figure 2.
Histopathological subtypes of intraductal papillary mucinous neoplasm (IPMN) of the pancreas and their typical mucin patterns. (A, B): IPMN of the intestinal type positive for MUC2. (C, D): IPMN of the pancreatobiliary type positive for MUC1. (E, F): IPMN of the oncocytic type showing positivity for MUC5. (G, H): IPMN of the gastric type, positive for MUC5.
Figure 3.
Figure 3.
Diagnostic algorithm in suspicious IPMN.
Figure 4.
Figure 4.
Therapeutic algorithm for IPMN. Abbreviations: BD-IPMN, branch duct IPMN; CT, computed tomography; EUS, endoscopic ultrasound; IPMN, intraductal papillary mucinous neoplasm; MD-IPMN, main duct IPMN; MRCP, magnetic resonance cholangiopancreatography; MRI, magnetic resonance imaging.
Figure 5.
Figure 5.
Clinical examples of IPMN. (A–C): BD-IPMN. (A): MRCP; (B): Intraoperative situs after enucleation; (C): Surgical specimen with BD-IPMN. (D–F): MD-IPMN. (D): CT with cystic head tumor and enlarged main pancreatic duct; (E): Mucin extrusion from a widely patent ampulla of Vater; (F): Surgical specimen with MD-IPMN. Abbreviations: BD-IPMN, branch duct IPMN; CT, computed tomography; IPMN, intraductal papillary mucinous neoplasm; MD-IPMN, main duct IPMN; MRCP, magnetic resonance cholangiopancreatography.
See this image and copyright information in PMC

References

    1. Kosmahl M, Pauser U, Anlauf M, et al. [Cystic pancreas tumors and their classification: Features old and new] Pathologe. 2005;26:22–30. - PubMed
    1. Ohhashi K, Murakami Y, Maruyama M, et al. Four cases of mucous secreting pancreatic cancer. Prog Digest Endosc. 1982;20:348–351.
    1. Morohoshi T, Kanda M, Asanuma K, et al. Intraductal papillary neoplasms of the pancreas. A clinicopathologic study of six patients. Cancer. 1989;64:1329–1335. - PubMed
    1. Klöppel G, Solcia E, Longnecker DS, et al. Second Edition. New York: Springer-Verlag; 1998. Histological Typing of Tumours of the Exocrine Pancreas: World Health Organization International Histological Classification of Tumours.
    1. Kosmahl M, Pauser U, Peters K, et al. Cystic neoplasms of the pancreas and tumor-like lesions with cystic features: A review of 418 cases and a classification proposal. Virchows Arch. 2004;445:168–178. - PubMed

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