Unraveling the enigma: A comprehensive review of solid pseudopapillary tumor of the pancreas

Abstract

Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly observed in young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and subsequent Wnt-signaling pathway activation are the leading molecular features. Accurate preoperative diagnosis often relies on imaging techniques and endoscopic biopsies. Surgical resection remains the mainstay treatment. Risk models, such as the Fudan Prognostic Index, show promise as predictive tools for assessing the prognosis of SPTP. Establishing three types of metachronous liver metastasis can be beneficial in tailoring individualized treatment and follow-up strategies. Despite advancements, challenges persist in understanding its etiology, establishing standardized treatments for unresectable or metastatic diseases, and developing a widely recognized grading system. This comprehensive review aims to elucidate the enigma by consolidating current knowledge on the epidemiology, clinical presentation, pathology, molecular characteristics, diagnostic methods, treatment options, and prognostic factors.

Comparison between solid pseudopapillary neoplasms of the pancreas and pancreatic ductal adenocarcinoma with cystic changes using computed tomography

“…In our study, a significant difference was found in peripancreatic invasion or distal metastasis between the two groups ( P = 0.006). Previous reports showed that size < 42.1 mm was one of the most common imaging features of aggressive SPNs[ 3 ]. In the present study, SPNs were more likely to have a larger size compared with PDAC with cystic changes (5.41 ± 2.89 cm vs 3.90 ± 1.24 cm, P = 0.017), and only 6.25% of SPNs showed peripancreatic invasion or distal metastasis, which is consistent with a previous study[ 3 ].…”

“…Previous reports showed that size < 42.1 mm was one of the most common imaging features of aggressive SPNs[ 3 ]. In the present study, SPNs were more likely to have a larger size compared with PDAC with cystic changes (5.41 ± 2.89 cm vs 3.90 ± 1.24 cm, P = 0.017), and only 6.25% of SPNs showed peripancreatic invasion or distal metastasis, which is consistent with a previous study[ 3 ]. We speculated that aggressive SPNs may be more likely to cause symptoms and be detected at a smaller size.…”

“…SPNs of the pancreas are low-grade malignant tumors that exhibit local metastasis or invasion in only 20% of cases[ 2 ]. Most SPNs are relatively indolent tumors that yield a great prognosis with complete surgical resection[ 3 ]. SPNs account for 1%-2% of all pancreatic neoplasms and 10%-15% of cystic pancreatic neoplasms[ 4 ].…”

Solid pseudopapillary neoplasm of the pancreas with hepatic metastases: problems and strategies

“…Moreover, in agreement with previous reports, the primary tumors from patients in this series were located in the body and tail of the pancreas. Liver metastasis of SPN can occur synchronously or metachronously ( 13 , 14 ). According to the literature, the rate of synchronous metastasis ranges between 0% and 4.3%, while the rate of metachronous metastasis ranges between 1.5% and 4.5% ( 14 ).…”