SPN is a rare neoplasm that affects mainly young women. Despite this feature, SPN have been seen in both genders, multiple races, and at a wide range of ages. The genetic mechanism behind the development of SPN is distinct from the more lethal ductal carcinoma of the pancreas. This difference is reflected in the favorable outcome for patients with SPN. Surgery is typically curative in patents with localized disease and possibly in patients with limited metastasis or local extension. No consensus exists on an effective systemic therapy. There are no reliable predictors for disease-specific mortality or recurrence in the minority of patients who develop aggressive disease.
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