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INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM IN PATIENTS UNDER AGE 50: TUMOR BIOLOGY, CLINICAL FEATURES, RECURRENCE RATES, AND SURVIVAL OUTCOMES
Vicente Morales-Oyarvide*1, Mari Mino-Kenudson2, Cristina R. Ferrone1, Andrew L. Warshaw1, Keith D. Lillemoe1, Ilaria Pergolini1, Marc Attiyeh3, Mohammad Al Efishat3, Neda Rezaee4, Ralph Hruban5,6, Jin He4, Matthew J. Weiss4, Peter Allen3, Christopher L. Wolfgang4, Carlos Fernandez-Del Castillo1
1Surgery, Massachusetts General Hospital, Boston, MA; 2Pathology, Massachusetts General Hospital, Boston, MA; 3Surgery, Memorial Sloan Kettering Cancer Center, New York, NY; 4Surgery, Johns Hospkins University School of Medicine, Baltimore, MD; 5Pathology, Memorial Sloan Kettering Cancer Center, New York, NY; 6Oncology, Memorial Sloan Kettering Cancer Center, New York, NY
Background: Intraductal papillary mucinous neoplasms (IPMN) of the pancreas are often diagnosed in the seventh decade of life. Whether clinical and pathological characteristics of young patients with resected IPMN differ from those in older patients remains unknown.
Methods: Retrospective study of 1,564 resected IPMN from the database of the Pancreatic Surgery Consortium. Patients were classified as young (<50 years) or older (≥50 years) based on their age at the time of surgery. We compared clinicopathological features and disease-specific survival between the two age groups using univariate and multivariate analyses.
Results: We identified 78 (5%) young patients and 1,486 (95%) older patients. Median age was 44 years (range 19-49) in the young group and 70 years (range 50-93) in older patients. There was no significant gender difference between the two age groups (49% men in each group). Young patients were significantly more likely to present with abdominal pain (54% vs. 34%, P=0.001) and less likely to present with jaundice (0% vs. 11%, P<0.001) or a history of diabetes mellitus (8% vs. 20%, P=0.006) compared with older patients. The proportion of branch-duct IPMN was similar between young and older patients (55% vs. 48%, P=0.331). IPMN in young patients were significantly more likely to be of intestinal-type (52% vs. 31%, P=0.017) and oncocytic-type (16% vs. 3%, P=0.006). Young age was not associated with non-invasive high-grade dysplasia (40% vs. 32%, P=0.177) but it was significantly associated with lower risk of invasive cancer (16% vs. 27%, P=0.031). This association persisted after adjusting for sex and IPMN type (OR 0.52, 95% CI 0.28-0.99, P=0.047). Among IPMN with an associated invasive carcinoma, young patients were less likely to have nodal metastasis (25% vs. 43%, P=0.216) and more likely to have a colloid type of invasive carcinoma (55% vs. 32%, P=0.189). Recurrence/progression rates of IPMN with and without associated invasive carcinoma were 13% for each age group; however, among patients that recurred, young patients were 5.4 times more likely to have reoperation (95% CI 2.10, 13.8, P<0.001) than older patients. In survival analysis, young patients had marginally significant improved 5-year (98.3% vs. 92.5%) and 10-year (98.3% vs. 89.9%) disease-specific survival compared with older patients (log-rank P=0.081).
Conclusion: Resection for IPMN is uncommon in patients under age 50, but when it occurs, these patients exhibit a distinct clinical and pathological profile. IPMN in the young are associated with higher prevalence of intestinal- and oncocytic-predominant epithelium, similar risk of non-invasive high-grade dysplasia as older patients but a lower risk of invasive cancer, and favorable disease-specific survival. These findings suggest IPMN arising in young and older patients may have underlying biological differences.
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