Solid Pseudopapillary Neoplasm of the Pancreas: Unfolding an Intriguing Condition

Abstract

Pancreatic cancer is one of the most lethal malignant neoplasms, with a 1-year survival rate after diagnosis of 24%, and a 5-year survival rate of only 9%. While this illustrates the behavior of its main histologic type – ductal adenocarcinoma, there are other histologic subtypes of pancreatic cancer that can harbor excellent prognosis. Solid pseudopapillary neoplasm, described as a rare low-grade malignant neoplasm by the World Health Organization, is the best example of that, having an overall 5-year survival rate of about 97%. Not only the prognosis, but everything about this entity is unique: its histogenesis, epidemiology, presentation, imaging characteristics, cytology features, immunohistochemical profile, and treatment. This explains the urge to improve our understanding about this entity and thus our ability to accurately recognize and manage it. Having this in mind, this article aims to summarize the most relevant topics regarding this entity.

Role of endoscopic ultrasound in the characterization of solid pseudopapillary neoplasm of the pancreas

“…Confirming previous reports, we found that the majority of patients were female with a 9:1 ratio[ 12 ]. Other features, age, size, and tumor appearance were similar, with no statistical significance.…”

Organ- or function-preservation surgery is recommended, but who is a candidate?

“…SPT frequently presents a single LMP neoplasm in the body and tail of the pancreas, which neither invades the surrounding structure nor causes biliary obstruction and metastasis, contributing to a 97% of an excellent 5-year OS rate. 17 All this may be a rationale for Dr. Shyr’s group to perform PHSS for SPT patients with/without ADP. 8 However, the literature review showed that recurrence or metastasis may occur after primary neoplasm resection, although it is a clinical situation of late recurrence, and it often occurs 8 to 16 years after surgery.…”

“…8 However, the literature review showed that recurrence or metastasis may occur after primary neoplasm resection, although it is a clinical situation of late recurrence, and it often occurs 8 to 16 years after surgery. 17 Since the median follow-up period of Dr. Shyr's group was only 67.7 months (72.8 months in SPT patients without ADP and particularly short [25.8 months] in SPT patients with ADP), 8 it may be inappropriate to claim that this PHSS could be recommended for SPT patients with ADP to avoid pancreatic insufficiency by the authors.…”

Solid pseudopapillary neoplasm – Management of an extremely rare case of pancreatic tumor in a young patient

“…Solid pseudopapillary tumor of the pancreas is a particularly rare entity, usually affecting young women in their second and third decades of life, but cases have also been reported among pediatric patients [5]. Often imaging incidentalomas, these neoplasms are frequently located in the head or tail of the pancreas, but literature also mentions retroperitoneal, mesenteric or adrenal as possible locations [4]. The clinical picture is poor and nonspecific, in about only a third of cases associating abdominal pain and discomfort.…”

“…[2] It is described in literature as having a low degree of malignancy, the metastases being exceptional. Surgery is curative in most cases [3,4].…”