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World Journal of Surgery

, Volume 41, Issue 7, pp 1871–1881 | Cite as

Solid Pseudopapillary Neoplasms of the Pancreas: A Surgical and Genetic Enigma

  • Leon Naar
  • Despoina-Amalia Spanomichou
  • Aikaterini MastorakiEmail author
  • Vassilios Smyrniotis
  • Nikolaos Arkadopoulos
Scientific Review

Abstract

Solid pseudopapillary neoplasms of the pancreas are rare tumors accounting for 1–2% of pancreatic exocrine neoplasms. This entity was first described by Dr. Frantz in 1959 and was defined by the World Health Organization in 1996 as “solid pseudopapillary tumor.” It is most often a benign neoplasm, but 10–15% of the cases are malignant. Over the past decades, the incidence of this tumor is increasing. However, many surgeons are still unfamiliar with this neoplasm and its unique characteristics, which can lead to pitfalls in the diagnosis and treatment. The correct diagnosis of SPNP is of utmost importance since it has a low malignant potential and with the appropriate treatment, patients have a long life expectancy. There are many genetic alterations, involving various signaling pathways that have been associated with SPNP and are very important in diagnosing the tumor. The cornerstone of SPNP treatment includes surgical excision of the tumor, preserving as much pancreatic tissue as possible. We review the information in the literature regarding more organ-preserving techniques and possible clinical features that might indicate a malignant potential, thus demanding a more radical intraoperative excision.

Keywords

Androgen Receptor Familial Adenomatous Polyposis Distal Pancreatectomy Solid Pseudopapillary Neoplasm Primary Pancreatic Tumor 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

Compliance with ethical standards

Conflict of interest

None.

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Copyright information

© Société Internationale de Chirurgie 2017

Authors and Affiliations

  • Leon Naar
    • 1
  • Despoina-Amalia Spanomichou
    • 1
  • Aikaterini Mastoraki
    • 1
    Email author
  • Vassilios Smyrniotis
    • 1
  • Nikolaos Arkadopoulos
    • 1
  1. 1.4th Department of SurgeryAthens University Medical School, ATTIKON University HospitalAthensGreece

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