Purpose The incidence of solid pseudopapillary neoplasm (SPN) of the pancreas is rising. Although the evidence for proper management is accumulating, we still lack diagnostic and therapeutic guidelines. In this paper, therefore, we propose an algorithm for diagnosis and treatment of this rare type of tumor. Methods A literature search was carried out on "Medline" and "Pubmed" databases to identify studies investigating the clinicopathologic features, pathogenesis, diagnostic, and differential diagnostic pathways, and surgical and adjuvant treatment options. Evidence from relevant published literature was completed with data of six patients treated with SPN in our institution. Results This study included case series and retrospective reviews only, since no higher level of evidence exists in the relevant literature. The articles emphasized that preoperative diagnosis is desirable to set up a precise plan for surgical treatment. Further, an R0 organ-sparing resection for primary SPN and an en bloc resection of locally advanced SPN are advised, while resection of synchronous as well as metachronous distant metastases is strongly advocated for this rare type of pancreatic cancer. The role of adjuvant chemo- or radiotherapy still needs to be defined. Finally, a diagnostic and therapeutic algorithm is devised in this paper to aid proper management of SPN. Conclusion Current recommendations for treatment of SPN of the pancreas rely mainly on case series as single institutional experiences and retrospective reviews. Although the level of evidence is relatively low, the way of management discussed above is likely to provide an excellent prognosis in typically young patients with SPN.
- Solid pseudopapillary neoplasm
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