Solid pseudopapillary tumor of the pancreas. A systematic review of clinical, surgical and oncological characteristics of 1384 patients underwent pancreatic surgery

Background: Pancreatic solid pseudopapillary tumors (SPTs) are rare clinical entity, with low malignancy and still unclear pathogenesis. They account for less than 2% of exocrine pancreatic neoplasms. This study aimed to perform a systematic review of the main clinical, surgical and oncological characteristics of pancreatic SPTs. Data sources: MEDLINE/PubMed, Web of Science and Scopus databases were systematically searched for the main clinical, surgical and oncological characteristics of pancreatic SPTs up to April 2021, in accordance with the preferred reporting items for systematic reviews and meta-analyses (PRISMA) standards. Primary endpoints were to analyze treatments and oncological outcomes. Results: A total of 823 studies were recorded, 86 studies underwent full-text reviews and 28 met inclusion criteria. Overall, 1384 patients underwent pancreatic surgery. Mean age was 30 years and 1181 patients (85.3%) were female. The most common clinical presentation was non-specific abdominal pain (52.6% of cases). Mean overall survival was 98.1%. Mean recurrence rate was 2.8%. Mean follow-up was 4.2 years. Conclusions: Pancreatic SPTs are rare, and predominantly affect young women with unclear pathogenesis. Radical resection is the gold standard of treatment achieving good oncological impact and a favorable prognosis in a yearly life-long follow-up.