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

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.

First robotic pylorus preserving pancreaticoduodenectomy for Frantz's tumour in an adolescent girl

“…[ 2 ] Patients typically present with abdominal pain or lump although some patients may remain asymptomatic until detected incidentally on clinical examination or radiological investigations. [ 3 ] Solid pseudopapillary tumours may appear in any part of the pancreas but are more likely to be found in the distal body and tail as also noted in our study. Our patient has SPT involving head and uncinate process of pancreas.…”

“…However, when imaging is not confirmatory, endoscopic ultrasound-guided cytology or biopsy can be done to confirm before surgery. [ 2 3 5 ] SPT is generally considered as a tumour with low malignant potential. Although resection of the tumour provides a 5-year survival rate more than 95%, local recurrence or distant metastases can occur.…”

Risk Factors of the Recurrences of Pancreatic Solid Pseudopapillary Tumors: A Systematic Review and Meta-analysis

“…Although SPTs have typical radiological features, some of them were still preoperatively misdiagnosed for pancreatic cystic neoplasms 23 . As for the confusion between pancreatic SPTs and cystic neoplasms, histological differential diagnosis is crucial 30 . Atypical imaging features including vascular invasion and invasion into surrounding pancreatic parenchyma, peripancreatic tissues and adjacent organs 31 are considered to be relevant with their aggressive behavior, which may cause the tumors to be prone to recur.…”

Solid Pseudopapillary Neoplasm of the Pancreas: Clinicopathologic Feature, Risk Factors of Malignancy, and Survival Analysis of 53 Cases from a Single Center

“…Operational style depended on tumor's size, tumor's location, and intraoperative frozen section examination. Due to the low malignancy of tumor, organ-preserving operation should be performed whenever feasible [21]. For 3 cases of pylorus-preserving pancreaticoduodenectomy, it was found that tumor invaded pancreatic parenchyma or duodenum in the intraoperative frozen section examination, so pylorus-preserving pancreaticoduodenectomy was indicated.…”