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

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 whe...

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Veröffentlicht in:Cancers 2021-01, Vol.13 (2), p.252
Hauptverfasser: Piombino, Eliana, Broggi, Giuseppe, Barbareschi, Mattia, Castorina, Sergio, Parenti, Rosalba, Bartoloni, Giovanni, Salvatorelli, Lucia, Magro, Gaetano
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container_start_page 252
container_title Cancers
container_volume 13
creator Piombino, Eliana
Broggi, Giuseppe
Barbareschi, Mattia
Castorina, Sergio
Parenti, Rosalba
Bartoloni, Giovanni
Salvatorelli, Lucia
Magro, Gaetano
description 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. 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 " " overgrowth and 1 recurrent and 2 primary tumors contained a minority of " " components. 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. 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.
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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 " " overgrowth and 1 recurrent and 2 primary tumors contained a minority of " " components. 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. 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.</description><identifier>ISSN: 2072-6694</identifier><identifier>EISSN: 2072-6694</identifier><identifier>DOI: 10.3390/cancers13020252</identifier><identifier>PMID: 33445443</identifier><language>eng</language><publisher>Switzerland: MDPI AG</publisher><subject>Age ; Biopsy ; CD34 antigen ; Cytoplasm ; Dermatofibrosarcoma protuberans ; Dermis ; Fasciitis ; Fibroids ; Immunohistochemistry ; Immunoreactivity ; Lesions ; Mesenchyme ; Morphology ; Musculoskeletal system ; Proteins ; Sarcoma ; Transcription factors ; Tumor suppressor genes ; Tumors</subject><ispartof>Cancers, 2021-01, Vol.13 (2), p.252</ispartof><rights>2021. This work is licensed under http://creativecommons.org/licenses/by/3.0/ (the “License”). 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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 " " overgrowth and 1 recurrent and 2 primary tumors contained a minority of " " components. 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. 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.</abstract><cop>Switzerland</cop><pub>MDPI AG</pub><pmid>33445443</pmid><doi>10.3390/cancers13020252</doi><orcidid>https://orcid.org/0000-0003-2576-6523</orcidid><orcidid>https://orcid.org/0000-0002-1614-4696</orcidid><oa>free_for_read</oa></addata></record>
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source PubMed Central Open Access; MDPI - Multidisciplinary Digital Publishing Institute; EZB-FREE-00999 freely available EZB journals; PubMed Central
subjects Age
Biopsy
CD34 antigen
Cytoplasm
Dermatofibrosarcoma protuberans
Dermis
Fasciitis
Fibroids
Immunohistochemistry
Immunoreactivity
Lesions
Mesenchyme
Morphology
Musculoskeletal system
Proteins
Sarcoma
Transcription factors
Tumor suppressor genes
Tumors
title 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
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