Solid pseudopapillary neoplasms of the pancreas are rare tumors accounting for 1-2% of pancreatic exocrine neoplasms. This entity was first described by Dr. Frantz in 1959 and was defined by the World Health Organization in 1996 as "solid pseudopapillary tumor." It is most often a benign neoplasm, but 10-15% of the cases are malignant. Over the past decades, the incidence of this tumor is increasing. However, many surgeons are still unfamiliar with this neoplasm and its unique characteristics, which can lead to pitfalls in the diagnosis and treatment. The correct diagnosis of SPNP is of utmost importance since it has a low malignant potential and with the appropriate treatment, patients have a long life expectancy. There are many genetic alterations, involving various signaling pathways that have been associated with SPNP and are very important in diagnosing the tumor. The cornerstone of SPNP treatment includes surgical excision of the tumor, preserving as much pancreatic tissue as possible. We review the information in the literature regarding more organ-preserving techniques and possible clinical features that might indicate a malignant potential, thus demanding a more radical intraoperative excision.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.