Solid Pseudopapillary Neoplasms of the Pancreas: A Review

Abstract

Solid Pseudopapillary Neoplasms (SPN) of the pancreas is rare tumors found throughout the pancreas composed of solid and cystic components. Since their description by Frantz, SPNs have been shown to comprise 1-2% of pancreatic tumors. These neoplasms predominantly affect young females in the second and third decades of life. Little is known about the pathogenesis of these tumors, although there is suggestion of a neuroendocrine origin or relationship with sex hormone receptors. The mainstay of treatment continues to be surgical resection, even in patients who present with locally advanced or metastatic disease. SPNs have low malignant potential with 5-year survival estimated as high as 95%, including those with malignant disease. We aim to review the current understanding regarding the diagnosis, management, and outcomes of patients with solid pseudopapillary neoplasms of the pancreas.