European Journal of Pediatric Surgery · 1999 · 20 citations · 0 references
MedicineOncologyPathologyHepatologyHistopathologyTumor PathologyHepatic PathologyHepatobiliary DiseaseHepatobiliary PathologyLiver CancerLiver DiseaseLiver ImagingHepatic MalignancyHepatic PathogenesisHepatic Tumor ImagingHepatic TumorsInflammatory Mimics Of MalignancyInflammatory PseudotumorLiver NeoplasmsPapillon-lefèvre Syndrome
A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process.