Published online Dec 15, 2017. doi: 10.4251/wjgo.v9.i12.497
Peer-review started: June 26, 2017
First decision: August 7, 2017
Revised: August 24, 2017
Accepted: November 3, 2017
Article in press: November 3, 2017
Published online: December 15, 2017
Solid pseudopapillary neoplasm (SPN), also known as Gruber-Frantz tumor, is a rare form of neoplasm that almost exclusively occurs in the pancreas and in young females. While the potential of malignancy is low for SPN, these tumors can mimic other diseases and require a meticulous investigation and a standard treatment by total surgical resection. We present an unusual case of SPN arising in the mesentery of a 40-year-old man with subsequent multiple metastases. Histopathological examination showed similar properties of the mesenteric neoplasm to those of SPN in pancreas. Although the mass was surgically removed, the patient died of recurrent disease 4 years after the initial presentation. We speculate that SPN originates from pancreatic progenitor cells. Further histopathological analyses are required for the prediction of SPN recurrence after resection.
Core tip: Solid pseudopapillary neoplasm (SPN) has been recognized by World Health Organization since 2010, and classified as a low malignant potential neoplasm. Such neoplasm is characterized by the presence of a mutation in the gene that encodes β-catenin. β-catenin is an important factor in the Wnt signaling pathway (β-catenin-dependent Wnt signaling). The identification of extrapancreatic SPN, especially in the mesentery, indicates a possible endoderm link between pancreatic progenitor cells and SPN cells.