title: A systematic review of solid-pseudopapillary neoplasms: are these rare lesions?
abstract: OBJECTIVE
  The aim of the study was to determine if there had been any change in the number of solid-pseudopapillary neoplasm (SPN) cases detected and their evaluation or management over time.
  
  
  METHODS
  A systematic review of SPN was performed of all articles published in English in PubMed and Scopus.
  
  
  RESULTS
  A total of 2744 patients with SPN were identified in 484 studies published between 1961 and 2012; 87.8% of the cases were reported between 2000 and 2012. A total of 2408 (87.8%) were females, and the mean age was 28.5 (SD, 13.7) years. The most common symptom was abdominal pain in 63.6% of the cases and incidentally detected in 38.1% of the cases. There were 2285 patients who underwent pancreatic resection. The mean tumor size was 8.6 (SD, 4.3) cm. Follow-up was reported for 1952 (90.5%) patients, with a mean follow-up of 36.1 (SD, 32.8) months. Disease-free survival was documented in 1866 (95.6%) patients with recurrence in 86 (4.4%) patients; the median time to recurrence was 50.5 months.
  
  
  CONCLUSIONS
  The number of SPNs reported in the literature has seen a 7-fold increase in the number of cases reported since 2000 compared with before. Solid-pseudopapillary neoplasms continue to be primarily found in young women and present with nonspecific symptoms. Surgery remains the mainstay of treatment with an excellent long-term prognosis.
authors.affiliation: From the *Division of Gastroenterology, †Department of Pathology, ‡Department of Radiology, Johns Hopkins University School of Medicine, §Department of Pathology, Sol Goldman Pancreatic Cancer Research Center, and ∥Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Joanna K
authors.initials: JK
authors.lastname: Law
authors.forename: Aadil
authors.initials: A
authors.lastname: Ahmed
authors.forename: Vikesh K
authors.initials: VK
authors.lastname: Singh
authors.forename: Venkata S
authors.initials: VS
authors.lastname: Akshintala
authors.forename: Matthew T
authors.initials: MT
authors.lastname: Olson
authors.forename: Siva P
authors.initials: SP
authors.lastname: Raman
authors.forename: Syed Z
authors.initials: SZ
authors.lastname: Ali
authors.forename: Elliot K
authors.initials: EK
authors.lastname: Fishman
authors.forename: Ihab
authors.initials: I
authors.lastname: Kamel
authors.forename: Marcia I
authors.initials: MI
authors.lastname: Canto
authors.forename: Marco
authors.initials: M
authors.lastname: Dal Molin
authors.forename: Robert A
authors.initials: RA
authors.lastname: Moran
authors.forename: Mouen A
authors.initials: MA
authors.lastname: Khashab
authors.forename: Nita
authors.initials: N
authors.lastname: Ahuja
authors.forename: Michael
authors.initials: M
authors.lastname: Goggins
authors.forename: Ralph H
authors.initials: RH
authors.lastname: Hruban
authors.forename: Christopher L
authors.initials: CL
authors.lastname: Wolfgang
authors.forename: Anne Marie
authors.initials: AM
authors.lastname: Lennon
mesh_terms: D000293:Adolescent; D000328:Adult; D002291:Carcinoma, Papillary / Q000175:diagnosis / Q000601:surgery*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D010180:Pancreatectomy / Q000379:methods*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000601:surgery*; D055815:Young Adult