6533b873fe1ef96bd12d5956

RESEARCH PRODUCT

Primitive Neuroectodermal Tumor (PNET) of the kidney: a case report.

Giorgio PomaraGirolamo MorelliCesare SelliMaria Giuseppa CuttanoMancini PFrancesco CappelloFrancesca Rappa

subject

AdultEwing's SarcomaPNETCancer ResearchPathologymedicine.medical_specialtyRenal PNETmedicine.medical_treatmentCD99Case Reportlcsh:RC254-282Diagnosis DifferentialCytokeratinImmunophenotypingSurgical oncologystem cellsGeneticsmedicineHumansNeuroectodermal Tumors Primitive Peripheralrhabdomyosarcoma tumorbusiness.industryEwing's sarcomalcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogensmedicine.diseaseNephrectomyKidney NeoplasmsOncologyPrimitive neuroectodermal tumorFemaleDifferential diagnosisbusinesscarcinogenesis

description

Abstract Background A case of Primitive Neuroectodermal Tumor (PNET) of the kidney in a 27-year-old woman is presented. Few cases are reported in the literature with a variable, nonspecific presentation and an aggressive behaviour. In our case, a radical nephrectomy with lymphadenectomy was performed and there was no residual or recurrent tumour at 24-month follow-up. Methods The surgical specimens were formalin-fixed and paraffin embedded. The sections were stained with routinary H&E. Immunohistochemistry was performed. Results The immunohistochemical evaluation revealed a diffuse CD99 positivity in the cytoplasm of the neoplastic cells. Pankeratin, cytokeratin AE1/AE3, vimentin, desmin, S100, cromogranin were negative. The clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype addressed the diagnosis towards primary PNET of kidney. Conclusions Since sometimes it is difficult to discriminate between PNET and Ewing's tumour, we reviewed the difficulties in differential diagnosis. These tumors have a common precursor but the stage of differentiation in which it is blocked is probably different. This could also explain their different biological behaviour and prognosis.

https://arpi.unipi.it/handle/11568/90580