Diagnosis and molecular aspects of solid-pseudopapillary neoplasms of the pancreas

摘要: Solid-pseudopapillary neoplasm of the pancreas (SPN) is an uncommon low-grade malignant occurring mostly in young women. In addition to its distinctive pathological appearance pseudopapillae with poorly cohesive neoplastic cells, rare variants exist raising differential diagnosis especially neuroendocrine neoplasms. The overall prognosis for patients SPNs excellent after surgical resection. Nevertheless, 10% cases may have behavior characterized by tumor recurrence and/or metastasis. Despite numerous studies, histogenesis this remains unclear. Distinctive molecular alterations such as presence CTNNB1 mutations are observed nearly all cases, while classically ductal adenocarcinoma, KRAS, TP53, and SMAD4, not SPNs, reinforcing distinct nature compared other pancreatic Recent transcriptional studies shown that activation Wnt/beta-catenin pathway these tumors associated upregulation genes belonging Notch, Hedgehog, androgen receptor signaling pathways.