胰腺实性假乳头状瘤的临床病理研究进展

Clinical and pathological characteristics of the solid pseudopapillary tumor of pancreas

胰腺实性假乳头状肿瘤是一种少见的低度恶性的胰腺实体肿瘤,好发于年轻女性,临床表现无特异性,而病理学特征明显,肿瘤呈囊实性结构,外被纤维包膜,典型表现为肿瘤细胞围绕纤维血管复层排列而形成假乳头状突起,少数肿瘤可出现侵袭性表现.肿瘤细胞表现出广泛的免疫标记表达.其发病机制与Wnt信号通路异常相关,β-catenin、P120-catenin、结肠腺瘤性息肉病基因APC、细胞周期素依赖的激酶抑制物p21和p27在其中发挥重要作用.

The solid pseudopapillary tumor of pancreas （SPT） is a rare pancreatic tumor with low malignant potential. It primarily affects young women. The clinical characteristic is nonspecific, but the pathological characteristic is distinct. The tumor consists of both cystic and solid component. The typical histopathological appearance of SPT is the pseudopapillary structure with a fibrovascular surrounded by several layers of epithelial cells.Only a few SPTs present invasive behaviors. The tumor cells express a wide range of immunohistochemical markers. The pathogenesis is associated with the Wnt signaling pathway, which is changed by β-catenin, Pl20-catenin, adenomatous polyposis coli gene（ APC）, the cyclin-dependent kinase inhibitors p21 and p27.