scholarly journals Wilms’ Tumor 1 (WT1): A Novel Immunomarker of Dermatofibrosarcoma Protuberans—An Immunohistochemical Study on a Series of 114 Cases of Bland-Looking Mesenchymal Spindle Cell Lesions of the Dermis/Subcutaneous Tissues

Cancers ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 252
Author(s):  
Eliana Piombino ◽  
Giuseppe Broggi ◽  
Mattia Barbareschi ◽  
Sergio Castorina ◽  
Rosalba Parenti ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Immunohistochemical Study ◽  
Wilms Tumor ◽  
Dermatofibrosarcoma Protuberans ◽  
Suppressor Gene ◽  
Cytoplasmic Staining ◽  
Primary Tumors ◽  
Subcutaneous Tissues ◽  
Wilms Tumor 1 ◽  
Cutaneous Leiomyomas

Purpose: to investigate the immunohistochemical expression and distribution of Wilms’ tumor 1 (WT1) (transcription factor produced by the tumor suppressor gene of the same name) in a series of 114 cases of bland-looking mesenchymal spindle cell lesions of the dermis/subcutaneous tissues to establish whether this immunomarker is differentially expressed in dermatofibrosarcoma protuberans (DFSP) versus its potential morphological mimickers. Methods: This retrospective multi-centric immunohistochemical study included 57 DFSP cases, 15 dermatofibromas, 5 deep fibrous histiocytomas, 8 neurofibromas, 5 spindle cell lipomas, 8 dermal scars, 6 nodular fasciitis, 5 cutaneous leiomyomas and 5 solitary fibrous tumors. Among the 57 DFSP cases, 11 were recurrent lesions; 2 non-recurrent cases exhibited an additional “fibrosarcomatous” overgrowth and 1 recurrent and 2 primary tumors contained a minority of “giant cell fibroblastoma” components. Results: Most DFSP (95% of cases) exhibited cytoplasmic staining for WT1; 11/11 residual/recurrent tumors showed diffuse and strong WT1 cytoplasmic immunoreactivity; apart from neurofibromas, WT1 expression was lacking in all the other cases studied. Conclusions: The cytoplasmic expression of WT1 may be exploitable as a complementary diagnostic immunomarker to CD34 in confirming the diagnosis of DFSP and to better evaluate the residual/recurrent tumor component.

A Role for the Wilms’ Tumor Protein WT1 in Organ Development

Physiology ◽  
2005 ◽  
Vol 20 (1) ◽  
pp. 54-59 ◽  
Author(s):  
Holger Scholz ◽  
Karin M. Kirschner
Keyword(s):  
Tumor Suppressor ◽  
Tumor Suppressor Gene ◽  
Wilms Tumor ◽  
Suppressor Gene ◽  
Gene Inactivation ◽  
Organ Development ◽  
Genitourinary System ◽  
Wilms Tumor 1 ◽  
Targeted Gene Inactivation

Wilms’ tumor (nephroblastoma) represents a unique example of an aberrant kidney formation that can result from mutations in a tumor suppressor gene, Wilms’ tumor 1 ( WT1). Targeted gene inactivation in mice testifies that WT1 is a master switch for the development of the genitourinary system and other organs.

Expression of WT-1, Bcl-2, and CD34 by Primary Renal Spindle Cell Tumors in Children

2004 ◽  
Vol 7 (6) ◽  
pp. 577-582 ◽  
Author(s):  
Lei Shao ◽  
D. Ashley Hill ◽  
Elizabeth J. Perlman
Keyword(s):  
Spindle Cell ◽  
Clear Cell ◽  
Immunohistochemical Study ◽  
Wilms Tumor ◽  
Expression Profiles ◽  
Potential Utility ◽  
Mesoblastic Nephroma ◽  
Immunohistochemical Markers ◽  
Synovial Sarcomas ◽  
Spindle Cell Tumors

The confident diagnosis of renal spindle cell tumors in children is often difficult. An immunohistochemical study of WT-1, Bcl-2, and CD34 was performed to determine their expression profiles and to assess the potential utility of these immunohistochemical markers in the differential diagnosis of 36 cases of renal spindle cell tumors of childhood. The cases included 11 stromal predominant Wilms tumors, 12 cellular mesoblastic nephromas, 9 clear cell sarcomas of the kidney (CCSK), and 4 monophasic synovial sarcomas. WT-1 was uniformly positive in primitive undifferentiated stromal Wilms tumors (6 of 6) and negative in the differentiating and differentiated stromal elements of Wilms tumors (0 of 5). WT-1 was also negative in cellular mesoblastic nephromas (0 of 12), CCSKs (0 of 12), and synovial sarcomas (0 of 4). Bcl-2 was expressed in all stromal Wilms tumors (11 of 11), all synovial sarcomas (4 of 4), some CCSKs (4 of 9), and none of the cellular mesoblastic nephromas (0 of 12). Although CD34 was absent in the tumor cells of all the tumors studied (0 of 36), CD34 immunohistochemistry nicely demonstrated the evenly distributed septal capillaries characteristic of CCSK in all 9 cases of this tumor. We conclude that a combination of WT-1 and Bcl-2 immunohistochemistry may aid in the distinction of stromal Wilms tumor, monophasic synovial sarcoma, cellular mesoblastic nephroma, and CCSK.

scholarly journals Immunohistochemical Expression of Wilms’ Tumor 1 Protein in Human Tissues: From Ontogenesis to Neoplastic Tissues

2019 ◽  
Vol 10 (1) ◽  
pp. 40
Author(s):  
Lucia Salvatorelli ◽  
Giovanna Calabrese ◽  
Rosalba Parenti ◽  
Giada Maria Vecchio ◽  
Lidia Puzzo ◽  
...  
Keyword(s):  
Tumor Suppressor ◽  
Tumor Suppressor Gene ◽  
Rna Splicing ◽  
Wilms Tumor ◽  
Suppressor Gene ◽  
Immunohistochemical Expression ◽  
Cortical Cells ◽  
Cytoplasmic Staining ◽  
N Terminus ◽  
Wt1 Protein

The human Wilms’ tumor gene (WT1) was originally isolated in a Wilms’ tumor of the kidney as a tumor suppressor gene. Numerous isoforms of WT1, by combination of alternative translational start sites, alternative RNA splicing and RNA editing, have been well documented. During human ontogenesis, according to the antibodies used, anti-C or N-terminus WT1 protein, nuclear expression can be frequently obtained in numerous tissues, including metanephric and mesonephric glomeruli, and mesothelial and sub-mesothelial cells, while cytoplasmic staining is usually found in developing smooth and skeletal cells, myocardium, glial cells, neuroblasts, adrenal cortical cells and the endothelial cells of blood vessels. WT1 has been originally described as a tumor suppressor gene in renal Wilms’ tumor, but more recent studies emphasized its potential oncogenic role in several neoplasia with a variable immunostaining pattern that can be exclusively nuclear, cytoplasmic or both, according to the antibodies used (anti-C or N-terminus WT1 protein). With the present review we focus on the immunohistochemical expression of WT1 in some tumors, emphasizing its potential diagnostic role and usefulness in differential diagnosis. In addition, we analyze the WT1 protein expression profile in human embryonal/fetal tissues in order to suggest a possible role in the development of organs and tissues and to establish whether expression in some tumors replicates that observed during the development of tissues from which these tumors arise.

scholarly journals H3K27me3-mediated silencing of Wilms Tumor 1 supports the proliferation of brain tumor cells harboring the H3.3K27M mutation

2017 ◽  
Author(s):  
Dong Fang ◽  
Haiyun Gan ◽  
Liang Cheng ◽  
Jeong-Heon Lee ◽  
Hui Zhou ◽  
...  
Keyword(s):  
Wilms Tumor ◽  
Suppressor Gene ◽  
Diffuse Intrinsic Pontine Glioma ◽  
Pontine Glioma ◽  
Mutant Proteins ◽  
Brain Tumor Cells ◽  
Wilms Tumor 1 ◽  
Global Reduction

AbstractThe lysine 27 to methionine mutation of histone H3.3 (H3.3K27M) is detected in over 75% of diffuse intrinsic pontine glioma (DIPG). The H3.3K27M mutant proteins inhibit H3K27 methyltransferase complex PRC2, resulting in a global reduction of tri-methylation of H3K27 (H3K27me3). Paradoxically, high levels of H3K27me3 were also detected at hundreds of genomic loci. However, it is not known how and why H3K27me3 is redistributed in DIPG cells. Here we show that lower levels of H3.3K27M mutant proteins at some genomic loci contribute to the retention of H3K27me3 peaks. But more importantly, Jarid2, a PRC2-associated protein, strongly correlates the presence of H3K27me3 and relieves the H3.3K27M-mediated inhibition in vivo and in vitro. Furthermore, we show that H3K27me3-mediated silencing of tumor suppressor gene Wilms Tumor 1 (WT1) supports the proliferation of DIPG cells and reaction of WT1 inhibits DIPG proliferation. Together, these studies reveal mechanisms whereby H3K27me3 is retained in the environment of global loss of this mark, and how persistence of this mark contributes to DIPG tumorigenesis.

Sign in / Sign up

Export Citation Format

Share Document