简介：Perivascularepithelioidcelltumor(PEComa)ofthepancreasisanunusualtumorderivingfrommesenchyma.ThispaperdescribedacaseofpancreaticPEComa,whichwasinitiallysuspectedasneuroendocrinecarcinomabybiopsy,andthereforesurgicaltreatmentwasrecommendedduetoundetermineddiagnosis.Examinationofthesurgicalspecimenunderamicroscopeshowedthatthetumorcell’smorphologywasepithelioidorspindle-shaped,andrangedinanestedpattern.Additionally,thesecellshadalargeextentofacidophiliccytoplasm,nomitoticfigures,andexpressedHMB-45,melan-p,andsmoothmuscleactinimmunohistochemically.PathologicalexaminationindicatedthatPEComaoriginatedfromthepancreas,butsymptomsrelatedtotuberoussclerosiswereabsent.SincePEComaisextremelyrareinthepancreas,itislikelytobeignoredindifferentialdiagnosis.Inconclusion,ourarticlehighlightedtheclinicopathologicalfeaturesofPEComa,andweconductedaliteraturereviewfocusingonPEComasoastodeepentheunderstandingofthistumortype.