Patients with a resected pancreatic mucinous cystic neoplasm have a better prognosis than patients with an intraductal papillary mucinous neoplasm: A large single institution series

James F Griffin; Andrew J Page; Georges J Samaha; Adrienne Christopher; Feriyl Bhaijee; Maryam K Pezhouh; Niek A Peters; Ralph H Hruban; Jin He; Martin A Makary; Anne Marie Lennon; John L Cameron; Christopher L Wolfgang; Matthew J Weiss

doi:10.1016/j.pan.2017.04.003

Patients with a resected pancreatic mucinous cystic neoplasm have a better prognosis than patients with an intraductal papillary mucinous neoplasm: A large single institution series

Pancreatology. 2017 May-Jun;17(3):490-496. doi: 10.1016/j.pan.2017.04.003. Epub 2017 Apr 11.

Affiliations

¹ Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
² Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
³ Department of Gastroenterology, The Johns Hopkins University School of Medicine, Baltimore, MD, USA.
⁴ Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address: mweiss5@jhmi.edu.

PMID: 28416122
DOI: 10.1016/j.pan.2017.04.003

Abstract

Background/objectives: Mucinous cystic neoplasms (MCNs) are rare pancreas tumors distinguished from intraductal papillary mucinous neoplasms (IPMNs) by the presence of ovarian-type stroma. Historical outcomes for MCNs vary due to previously ambiguous diagnostic criteria resulting in confusion with IPMNs. This study seeks to characterize and clarify the clinical features and long-term outcomes of MCNs versus IPMNs in the largest single-institution series of pathology-confirmed MCNs to date.

Methods: We compared 142 MCNs and 746 IPMNs resected at a single institution. MCNs were reviewed for confirmation of ovarian-type stroma and reclassified according to current WHO guidelines.

Results: MCNs presented almost exclusively in middle-aged women (median 47.5 years, 96.5% female) as solitary (100%), macrocystic (94.2%) lesions in the distal pancreas (92.1%). IPMNs were distributed equally by sex in an older population (median 69.0 years, 49.6% female) and favored the proximal pancreas (67.6%). Compared with IPMNs, MCNs were larger (4.2 cm vs 2.5 cm) and more often low-grade (71.1% vs 13.8%). Associated invasive carcinoma was less common in MCNs than in IPMNs (9.9% vs 32.4%). Surgical resection was curative for 100% of noninvasive MCNs. Patients with an MCN-associated invasive carcinoma had a much better prognosis than did patients with an IPMN-associated invasive carcinoma with 10-year disease-specific survival of 79.6% versus 27.2%, respectively.

Conclusion: MCNs have a stereotypical clinical profile that is readily distinguishable from IPMNs based on demographic features, imaging, and pathology. Most MCNs are noninvasive and curable with surgical resection. Prognosis remains excellent even for invasive disease with 10-year survival approaching 80% following resection.

Keywords: Intraductal papillary mucinous neoplasm (IPMN); Ovarian stroma; Pancreatic adenocarcinoma (PDAC); Pancreatic cystic neoplasms; Pancreatic mucinous cystic neoplasm (MCN).

Publication types

Comparative Study

MeSH terms

Adult
Age Factors
Aged
Cohort Studies
Female
Humans
Kaplan-Meier Estimate
Male
Middle Aged
Neoplasms, Cystic, Mucinous, and Serous / diagnostic imaging
Neoplasms, Cystic, Mucinous, and Serous / surgery*
Pancreatectomy
Pancreatic Neoplasms / diagnostic imaging
Pancreatic Neoplasms / surgery*
Papilloma, Intraductal / diagnostic imaging
Papilloma, Intraductal / surgery*
Prognosis
Retrospective Studies
Sex Factors
Survival Analysis
Treatment Outcome