关键词: 侵袭性纤维瘤病, 胰腺实性假乳头状肿瘤, β-catenin, 复发, APC

Abstract: The clinical manifestations,pathological morphological features,immunohistochemical staining results and molecular pathological alterations of one case of recurrent intra-abdominal AF complicated with pancreatic SPN were analyzed,and the relevant literature was reviewed.AF was composed of long spindle-shaped tumor cells with relatively consistent size and shape,arranged in fascicular or woven structures.The results of immunohistochemical staining:β-catenin (nuclear +).Most cases of AF are sporadic and are related to mutations in exon 3 of the CTNNB1 gene;a few cases are related to APC gene mutations.SPN was composed of a solid area and a pseudopapillary area.The pseudopapillary structure was formed by cells loosely arranged around the fibrovascular axis.The results of immunohistochemical staining:CK (+),Vimentin (+),β-catenin (nuclear +),CD10 (+),PR (+),Syn (+).Most cases of SPN have mutations in exon 3 of the CTNNB1 gene;in rare cases,gene mutations such as CDKN2A,TP53,and LRP1B may also occur.The simultaneous occurrence of these two tumors is very rare,and only individual case reports can be found in domestic and foreign literatures.More cases need to be accumulated to further explore the treatment and prognosis.

Key words: aggressive fibromatosis, solid pseudopapillary neoplasm of the pancreas, β-catenin, recurrent, APC