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Immunohistochemical Characterization of a Renal Nephroblastoma in a Trp53-mutant and Prolyl Isomerase 1-deficient Mouse.

Castiglioni V, De Maglie M, Queliti R, Rustighi A, Del Sal G, Radaelli E - J Toxicol Pathol (2013)

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: 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.

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.

No MeSH data available.


Related in: MedlinePlus

Kidney;biphasic nephroblastoma in a Trp53-mutant and prolyl isomerase1-deficient mouse. (A) The tumor is composed of neoplastic epithelial cells arranged insingle-layered tubules surrounded by neoplastic blastemal cells arranged in nests.Hematoxylin and eosin, 200×. (B) Both epithelial and blastemal cell populations arecharacterized by marked and diffuse nuclear immunoreactivity for Wilms’ tumor antigen 1.Wilms’ tumor antigen 1 immunohistochemistry (IHC), 200×. (C) Scant cytoplasmicimmunoreactivity for vimentin is observed in the stromal compartment separatingneoplastic cell populations. Vimentin IHC, 100×. (D) Neoplastic epithelial cells liningtubules diffusely express cytokeratin. Wide spectrum cytokeratin IHC, 100×. (E)Scattered tubules display FBP immunoreactivity, which is mainly concentrated along theluminal surface of neoplastic epithelial cells. FBP IHC, 200×. (F) Few scatteredneoplastic mesenchymal cells are diffusely positive for smooth muscle actin (SMA). SMAIHC, 200×. (G) A large number of Ki-67-positive neoplastic cells are evident in theblastemal compartment, whereas only scattered Ki-67-positive neoplastic epithelial cellsline the tubular structures. Ki-67 IHC, 200×. (H) Both epithelial and blastemal cellpopulations are characterized by marked and diffuse nuclear and cytoplasmicimmunoreactivity for β-catenin. β-catenin IHC, 200×.
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fig_001: Kidney;biphasic nephroblastoma in a Trp53-mutant and prolyl isomerase1-deficient mouse. (A) The tumor is composed of neoplastic epithelial cells arranged insingle-layered tubules surrounded by neoplastic blastemal cells arranged in nests.Hematoxylin and eosin, 200×. (B) Both epithelial and blastemal cell populations arecharacterized by marked and diffuse nuclear immunoreactivity for Wilms’ tumor antigen 1.Wilms’ tumor antigen 1 immunohistochemistry (IHC), 200×. (C) Scant cytoplasmicimmunoreactivity for vimentin is observed in the stromal compartment separatingneoplastic cell populations. Vimentin IHC, 100×. (D) Neoplastic epithelial cells liningtubules diffusely express cytokeratin. Wide spectrum cytokeratin IHC, 100×. (E)Scattered tubules display FBP immunoreactivity, which is mainly concentrated along theluminal surface of neoplastic epithelial cells. FBP IHC, 200×. (F) Few scatteredneoplastic mesenchymal cells are diffusely positive for smooth muscle actin (SMA). SMAIHC, 200×. (G) A large number of Ki-67-positive neoplastic cells are evident in theblastemal compartment, whereas only scattered Ki-67-positive neoplastic epithelial cellsline the tubular structures. Ki-67 IHC, 200×. (H) Both epithelial and blastemal cellpopulations are characterized by marked and diffuse nuclear and cytoplasmicimmunoreactivity for β-catenin. β-catenin IHC, 200×.

Mentions: Histologically, the renal parenchyma was completely effaced by a densely cellular, partiallyencapsulated, lobulated mass composed of a mixture of 2 main cell populations and supported bya minimal amount of stroma. The first population consisted of neoplastic epithelial cellsarranged in single-layered infolded tubules, which were occasionally filled by necrotic debris(Fig. 1AFig. 1.


Immunohistochemical Characterization of a Renal Nephroblastoma in a Trp53-mutant and Prolyl Isomerase 1-deficient Mouse.

Castiglioni V, De Maglie M, Queliti R, Rustighi A, Del Sal G, Radaelli E - J Toxicol Pathol (2013)

Kidney;biphasic nephroblastoma in a Trp53-mutant and prolyl isomerase1-deficient mouse. (A) The tumor is composed of neoplastic epithelial cells arranged insingle-layered tubules surrounded by neoplastic blastemal cells arranged in nests.Hematoxylin and eosin, 200×. (B) Both epithelial and blastemal cell populations arecharacterized by marked and diffuse nuclear immunoreactivity for Wilms’ tumor antigen 1.Wilms’ tumor antigen 1 immunohistochemistry (IHC), 200×. (C) Scant cytoplasmicimmunoreactivity for vimentin is observed in the stromal compartment separatingneoplastic cell populations. Vimentin IHC, 100×. (D) Neoplastic epithelial cells liningtubules diffusely express cytokeratin. Wide spectrum cytokeratin IHC, 100×. (E)Scattered tubules display FBP immunoreactivity, which is mainly concentrated along theluminal surface of neoplastic epithelial cells. FBP IHC, 200×. (F) Few scatteredneoplastic mesenchymal cells are diffusely positive for smooth muscle actin (SMA). SMAIHC, 200×. (G) A large number of Ki-67-positive neoplastic cells are evident in theblastemal compartment, whereas only scattered Ki-67-positive neoplastic epithelial cellsline the tubular structures. Ki-67 IHC, 200×. (H) Both epithelial and blastemal cellpopulations are characterized by marked and diffuse nuclear and cytoplasmicimmunoreactivity for β-catenin. β-catenin IHC, 200×.
© Copyright Policy - open-access
Related In: Results  -  Collection

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Show All Figures
getmorefigures.php?uid=PMC3921926&req=5

fig_001: Kidney;biphasic nephroblastoma in a Trp53-mutant and prolyl isomerase1-deficient mouse. (A) The tumor is composed of neoplastic epithelial cells arranged insingle-layered tubules surrounded by neoplastic blastemal cells arranged in nests.Hematoxylin and eosin, 200×. (B) Both epithelial and blastemal cell populations arecharacterized by marked and diffuse nuclear immunoreactivity for Wilms’ tumor antigen 1.Wilms’ tumor antigen 1 immunohistochemistry (IHC), 200×. (C) Scant cytoplasmicimmunoreactivity for vimentin is observed in the stromal compartment separatingneoplastic cell populations. Vimentin IHC, 100×. (D) Neoplastic epithelial cells liningtubules diffusely express cytokeratin. Wide spectrum cytokeratin IHC, 100×. (E)Scattered tubules display FBP immunoreactivity, which is mainly concentrated along theluminal surface of neoplastic epithelial cells. FBP IHC, 200×. (F) Few scatteredneoplastic mesenchymal cells are diffusely positive for smooth muscle actin (SMA). SMAIHC, 200×. (G) A large number of Ki-67-positive neoplastic cells are evident in theblastemal compartment, whereas only scattered Ki-67-positive neoplastic epithelial cellsline the tubular structures. Ki-67 IHC, 200×. (H) Both epithelial and blastemal cellpopulations are characterized by marked and diffuse nuclear and cytoplasmicimmunoreactivity for β-catenin. β-catenin IHC, 200×.
Mentions: Histologically, the renal parenchyma was completely effaced by a densely cellular, partiallyencapsulated, lobulated mass composed of a mixture of 2 main cell populations and supported bya minimal amount of stroma. The first population consisted of neoplastic epithelial cellsarranged in single-layered infolded tubules, which were occasionally filled by necrotic debris(Fig. 1AFig. 1.

Bottom Line: 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.

View Article: PubMed Central - PubMed

Affiliation: 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.

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.

No MeSH data available.


Related in: MedlinePlus