title: Immunohistochemical Characterization of a Renal Nephroblastoma in a Trp53-mutant and Prolyl Isomerase 1-deficient Mouse.
abstract: A nephroblastoma is a tumor arising from metanephric blastema occurring in childhood. Among laboratory rodents, nephroblastoma has been frequently reported in rats, but it remains exceedingly rare in mice. The present work describes a nephroblastoma in a young mouse homozygous for the specific Trp53 R172H point mutation coupled with targeted deletion of the Pin1 gene. The affected kidney was effaced by a biphasic tumor with an epithelial component arranged in tubules surrounded by nests of blastemal cells. Immunohistochemically, the neoplasm was diffusely positive for Wilms' tumor antigen. The epithelial component expressed markers of renal tubular differentiation including wide-spectrum cytokeratin, E-cadherin and folate-binding protein. Furthermore, the neoplasm exhibited a high proliferative index and diffuse nucleocytoplasmic β-catenin expression. Based on histological and immunohistochemical features, a diagnosis of nephroblastoma potentially associated with Trp53 loss and oncogenic β-catenin activation has been proposed.
authors: Dipartimento di Scienze Veterinarie e Sanità Pubblica Veterinaria (DIVET), Facolta di Medicina Veterinaria, Università degli Studi di Milano, Via Celoria, 10, 20133 Milano, Italy ; Mouse & Animal Pathology Lab, Fondazione Filarete, Viale Ortles, 22/4, 20139 Milano, Italy.
authors: Vittoria
authors: V
authors: Castiglioni
authors: Mouse & Animal Pathology Lab, Fondazione Filarete, Viale Ortles, 22/4, 20139 Milano, Italy.
authors: Marcella
authors: M
authors: De Maglie
authors: Centro Ricerche Bracco, Bracco Imaging Spa, via Ribes 5, 10010 Colleretto Giacosa (TO), Italy.
authors: Roberta
authors: R
authors: Queliti
authors: Laboratorio Nazionale CIB (LNCIB), Area Science Park, 34149 Trieste, Italy ; Dipartimento di Scienze della Vita, Università degli Studi di Trieste, 34127 Trieste, Italy.
authors: Alessandra
authors: A
authors: Rustighi
authors: Laboratorio Nazionale CIB (LNCIB), Area Science Park, 34149 Trieste, Italy ; Dipartimento di Scienze della Vita, Università degli Studi di Trieste, 34127 Trieste, Italy.
authors: Giannino
authors: G
authors: Del Sal
authors: Dipartimento di Scienze Veterinarie e Sanità Pubblica Veterinaria (DIVET), Facolta di Medicina Veterinaria, Università degli Studi di Milano, Via Celoria, 10, 20133 Milano, Italy ; Mouse & Animal Pathology Lab, Fondazione Filarete, Viale Ortles, 22/4, 20139 Milano, Italy.
authors: Enrico
authors: E
authors: Radaelli
keywords: immunohistochemistry; kidney; mouse; nephroblastoma; pathogenesis