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

Core Tip

Core Tip: Solid pseudopapillary tumor of the pancreas (SPTP) is a rare neoplasm predominantly affecting young females. Pathologically, CTNNB1 mutations, β-catenin nuclear accumulation, and Wnt signaling pathway activation are key molecular features. Accurate preoperative diagnosis relies on imaging and endoscopic biopsies. Surgical resection is the main treatment, and prognostic models like Fudan Prognostic Index aid in prognosis assessment. Challenges in understanding its etiology, establishing treatments for unresectable/metastatic disease, and developing a standardized grading system persist. This comprehensive review aims to consolidate current knowledge on epidemiology, clinical presentation, pathology, molecular features, and treatment options for SPTP.