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Cystic Pancreatic Endocrine Neoplasms: A Distinct Tumor Type?
Liliana G Bordeianou, Parsia A Vagefi, Dushyant Sahani, Vikram Deshpande, Elena Rakhlin, Andrew L Warshaw, Carlos Fernández -del Castillo
Massachusetts General Hospital, Boston, MA

Objectives: Cystic Pancreatic Endocrine Neoplasms (CPENs) are considered rare, and their behavior is thought to be similar to that of solid PENs. This study aims to describe the characteristics of CPENs in a large patient cohort.
Design: Retrospective review from 1977 to 2006.
Setting: Massachusetts General Hospital, a tertiary care center.
Patients: 29 patients (51% male, mean age 53) underwent resection for CPENs, and were compared to 138 patients with solid PENs.
Main Outcome Measures: Differences in clinical presentation, pathological and radiographic features, and survival.
Results: CPENs comprised 18% of pancreatic endocrine neoplasms(29/167). 10 (34%) were purely cystic and 19 (66%) were partially cystic. Compared to solid PENs, CPENs were larger (49mm vs. 23.5mm, P<0.05), more likely symptomatic (73% vs. 45%; P<0.05) , and more likely to be non-functional (80% vs. 50% ; P<0.05). They expressed synaptophysin (100%), chromogranin (82%), pancreatic polypeptide (74%), and CK-19 (24%). MEN-1 was 3.5 times more common in cystic PENs than in solid tumors (21% vs. 6%, P<0.05). No significant difference was found regarding location, propensity for metastasis, invasion, or 5- year survival ( 87% vs. 77%, p =0.38).
Conclusion: This series, the largest report of CPENs in the current literature, shows that these neoplasms are more common than previously thought. CPENs are larger and more likely to be symptomatic then solid PENs. They are also more likely to be associated with MEN-1 and to be non-functional, suggesting they may be a distinct tumor type.


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