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Int J Clin Exp Pathol 2013;6(6):1051-1059

Original Article
Solid-pseudopapillary neoplasm (SPN) of the pancreas: case series and literature review
on an enigmatic entity

Nikolaos Vassos, Abbas Agaimy, Peter Klein, Werner Hohenberger, Roland S Croner

Department of Surgery, University Hospital, Erlangen, Germany; Institute of Pathology, University Hospital, Erlangen, Germany. Equally
contributed.

Received March 19, 2013; Accepted April 23, 2013; Epub May 15, 2013; Published June 1, 2013

Abstract: Solid pseudopapillary neoplasm (SPN) of the pancreas is a rare tumor which typically affects young women without significant
clinical symptoms. SPN usually shows an indolent behavior and only rare cases recur and/or metastasize after complete resection. We report
our experience with four cases of SPN of the pancreas. All four patients were female with an age range of 15-42 years (mean age: 24.5 years).
Two patients presented with abdominal pain, one with abdominal mass and one with acute abdominal signs following blunt trauma. Tumor’s
size ranged from 1 to 16 cm (mean size: 5.5 cm). Two tumors were diagnosed preoperatively through percutaneous core needle biopsy and
two underwent surgery without preoperative diagnosis because of high suspicion of SPN based on clinical and radiological findings. By
immunohistochemistry, all cases stained strongly for vimentin, progesterone-receptor and beta-catenin (nuclear) and variably with pankeratin
and neuroendocrine markers. The proliferation index (Ki-67) was <2% in all cases. After a median follow-up of 40 months (range: 24-57
months), all patients were alive with no evidence of recurrence or metastatic disease. In conclusion, SPN of the pancreas should be
considered in the differential diagnosis of any solid and partly cystic pancreatic or upper abdominal mass, particularly in young females. SPN
possesses a low malignant potential and complete surgical resection with clear margins is the treatment of choice. Following R0 resection,
SPN has an excellent prognosis. (IJCEP1303045).

Keywords: Solid pseudopapillary neoplasm, Frantz tumor, pancreas, SPN

Address correspondence to: Dr. Nikolaos Vassos, Department of Surgery, University Hospital Erlangen, Krankenhausstraße 12, D-91054
Erlangen, Germany. Tel: +49-9131-85-33296; Fax: +49-9131-85-36294; E-mail: nikolaos.vassos@uk-erlangen.de