title: Pediatric gastrointestinal PEComas: a diagnostic challenge.
abstract: Perivascular epithelioid cell tumors (PEComas) in the pediatric population are very rare and frequently arise in the gastrointestinal tract. These tumors are characterized by variable morphological and immunohistochemical features, which may pose significant problems in differential diagnosis with other gastrointestinal mesenchymal tumors in pediatric patients. In presenting an unusual pediatric case of gastrointestinal PEComa, we also discuss the possible differential diagnosis of this rare entity.
authors.affiliation: 1  Department of Medicine-DIMED, General Pathology & Cytopathology Unit, University of Padova, Padova, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Pizzi
authors.forename: Ida
authors.initials: I
authors.lastname: di Lorenzo
authors.forename: Emanuele S
authors.initials: ES
authors.lastname: d'Amore
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism*; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis* / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: PEComa; SOX10; clear cell sarcoma; gastrointestinal mesenchymal tumors; pediatric pathology