title: Primary primitive peripheral neuroectodermal tumor of the prostate. Immunophenotypic and molecular study of a case.
abstract: A case of primitive peripheral neuroectodermal tumor arising in the prostate gland of a 31-year-old man and first diagnosed through a biopsy is reported. Microscopically, the tumor was made up of solid nests and sheets of small round cells, and it was difficult to distinguish the neoplasm from other small round cell tumors, such as small cell carcinoma, rhabdomyosarcoma, or malignant lymphoma. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, vimentin, neuron-specific enolase, and synaptophysin. The neoplasm was excised by a radical surgical procedure preceded by chemotherapy and radiation therapy. The morphologic diagnosis of the prostatectomy specimen was complemented by molecular analysis performed on viable microdissected tissue obtained from formalin-fixed, paraffin-embedded tumor sections. Polymerase chain reaction and sequencing assessment showed the presence of EWS/FLI1 type 2 chimeric transcript, confirming the diagnosis of peripheral primitive neuroectodermal tumor. To our knowledge, this is the first description of a primary peripheral primitive neuroectodermal tumor in the prostate gland.
authors.affiliation: Dipartimento di Patologia, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milano, Italy. colecchia@istitutotumori.mi.it
authors.forename: Maurizio
authors.initials: M
authors.lastname: Colecchia
authors.forename: GianPaolo
authors.initials: G
authors.lastname: Dagrada
authors.forename: Pietro Luigi
authors.initials: PL
authors.lastname: Poliani
authors.forename: Antonella
authors.initials: A
authors.lastname: Messina
authors.forename: Silvana
authors.initials: S
authors.lastname: Pilotti
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis / Q000235:genetics / Q000276:immunology; D020732:Cytogenetic Analysis / Q000379:methods*; D052138:Genes, Neoplasm / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping / Q000379:methods*; D008297:Male; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics*; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011471:Prostatic Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics*; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; C106031:EWS-FLI fusion protein; D015514:Oncogene Proteins, Fusion; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D014157:Transcription Factors