Pancreatic solid-pseudopapillary tumors in children: clinicopathological analyses of 8 cases
ZHAO Xue-yan
ZHANG Tao-tao
JING Cai-ping
DENG Yuan
Abstract：Purpose To investigate the clinicopahological characteristics and prognosis in 8 cases of solid pseudopapillary tumor of the pancreas in children, and to improve the understanding of the disease. Methods Eight cases of SPPT were observed with conventional histopathologic analysis and immunohistochemistry from 2007 to 2017. Retrospective review was conducted with clinical data and the related literature was reviewed to analyze the histogenesis and biological behavior of the disease. Results Tumors were cystic and solid or solid nudules with clear borderline. Microscopic evaluation revealed that the tumor was composed of medium-sized cells with different proportions of solid, papillary and cystic structures. The papillary surface was covered with multilayer tumor cells; most tumor nuclei were away from the fibrovascular axis, similar to the pseudorossette structure. Immunohistochemically, the tumors were usually positive for a-AT, vimentin, β-caternin, claudin-5, PR and CD10, but usually negative for E-cadherin; the tumors could express endocrine markers, such as CD56, NSE, Syn and CgA. Ki-67 index was low. Conclusions SPPT is a tumor with low malignancy of the pancreas in children; the prognosis is better if the tumor has been completely resected even with the infiltration of tumor surrounding tissue. The tumor may originate from the pancreatic primordial pluripotent stem cell. Molecular expression of SPPT is mainly focused on Wnt/beta-caternin and Notch signaling pathways, leading to beta-caternin expression in the nucleus and cytoplasm, and loss expression of E-cadherin. Sex hormones may play an important role in SPPT growth. Typical histological features and expression of some immunohistochemical markers (α-AT, vimentin, β-caternin, Claudin-5, PR, CD10 and E-cadherin) can help diagnosis and differential diagnosis for SPPT.
Keywords：Pancreatic tumorSolid pseudopapillary tumorClinical pathologyImmunohistochemistry
Publication Date：2018-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 447-450 )