title: Peripheral primitive neuroectodermal tumor within the spinal epidural space.
abstract: A case of an epidural spinal peripheral primitive neuroectodermal tumor (pPNET) in a 13-year-old girl is presented. The tumor was disseminated at the moment of diagnosis, thus only diagnostic oligobiopsy of the epidural mass was performed. Histologically the tumor was composed of small round blue cells. The neoplastic cells expressed MIC2 and features of neural differentiation on immunohistochemical staining (neuron-specific enolase, synaptophysin and NCAM positivity). Fluorescent in situ hybridization (FISH) analysis was performed for the final diagnosis and the translocation t(11;22)(q24;q12) was detected. The present case emphasizes the usefulness of FISH in differential diagnosis of tumors, especially when only routinely fixed material is available.
authors.affiliation: Department of Pathology, Medical University of Gdansk, Poland. eczis@wp.pl
authors.forename: E
authors.initials: E
authors.lastname: Izycka-Swieszewska
authors.forename: J
authors.initials: J
authors.lastname: Stefanowicz
authors.forename: M
authors.initials: M
authors.lastname: Debiec-Rychter
authors.forename: R
authors.initials: R
authors.lastname: Rzepko
authors.forename: J
authors.initials: J
authors.lastname: Borowska-Lehman
mesh_terms: D000071443:12E7 Antigen; D000293:Adolescent; D015703:Antigens, CD / Q000378:metabolism; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D015815:Cell Adhesion Molecules / Q000378:metabolism; D002574:Cervical Vertebrae; D002869:Chromosome Aberrations; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics; D002892:Chromosomes, Human, Pair 22 / Q000235:genetics; D003937:Diagnosis, Differential; D015174:Epidural Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D004824:Epidural Space / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D019006:Neural Cell Adhesion Molecules / Q000378:metabolism; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D016708:Synaptophysin / Q000378:metabolism; D013904:Thoracic Vertebrae; D016896:Treatment Outcome
chemical_list: D000071443:12E7 Antigen; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D005904:Glial Fibrillary Acidic Protein; D019006:Neural Cell Adhesion Molecules; D016708:Synaptophysin; D010751:Phosphopyruvate Hydratase