A systematic review of solid-pseudopapillary neoplasms: Are these rare lesions?

Joanna K. Law, Aadil Ahmed, Vikesh K. Singh, Venkata S. Akshintala, Matthew T. Olson, Siva P. Raman, Syed Z. Ali, Elliot K. Fishman, Ihab Kamel, Marcia I. Canto, Marco Dal Molin, Robert A. Moran, Mouen A. Khashab, Nita Ahuja, Michael Goggins, Ralph H. Hruban, Christopher L. Wolfgang, Anne Marie Lennon

Research output: Contribution to journalReview articlepeer-review

145 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)331-337
Number of pages7
Issue number3
StatePublished - Apr 2014


  • Cyst
  • Pancreas
  • Review
  • Solid-pseudopapillary neoplasm
  • Systematic review

ASJC Scopus subject areas

  • Internal Medicine
  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Endocrinology


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