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肾母细胞瘤1（WT1）：隆突性皮肤纤维肉瘤的一种新型免疫标志物——对114例外观平淡的真皮/皮下组织间质性梭形细胞病变的免疫组织化学研究

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.

作者信息

Piombino Eliana, Broggi Giuseppe, Barbareschi Mattia, Castorina Sergio, Parenti Rosalba, Bartoloni Giovanni, Salvatorelli Lucia, Magro Gaetano

机构信息

Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Azienda Ospedaliero-Universitaria "Policlinico Vittorio Emanuele", Anatomic Pathology, School of Medicine, University of Catania, 95123 Catania, Italy.

Pathology Unit, Department of Clinical Services, Santa Chiara Hospital, 38122 Trento, Italy.

出版信息

Cancers (Basel). 2021 Jan 12;13(2):252. doi: 10.3390/cancers13020252.

DOI:10.3390/cancers13020252
PMID:33445443
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7826654/
Abstract

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 "" overgrowth and 1 recurrent and 2 primary tumors contained a minority of "" 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.

摘要

目的

研究威尔姆斯瘤1（WT1）（由同名肿瘤抑制基因产生的转录因子）在114例外观平淡的真皮/皮下组织间质性梭形细胞病变中的免疫组化表达及分布情况，以确定该免疫标志物在隆突性皮肤纤维肉瘤（DFSP）及其潜在形态学模仿者中是否存在差异表达。

方法

这项回顾性多中心免疫组化研究包括57例DFSP病例、15例皮肤纤维瘤、5例深部纤维组织细胞瘤、8例神经纤维瘤、5例梭形细胞脂肪瘤、8例皮肤瘢痕、6例结节性筋膜炎、5例皮肤平滑肌瘤和5例孤立性纤维瘤。在57例DFSP病例中，11例为复发病变；2例非复发病例表现出额外的“过度生长”，1例复发病例和2例原发性肿瘤含有少数“”成分。

结果

大多数DFSP（95%的病例）表现出WT1的细胞质染色；11/11例残留/复发性肿瘤显示WT1细胞质免疫反应弥漫且强烈；除神经纤维瘤外，所有其他研究病例均缺乏WT1表达。

结论

WT1的细胞质表达可作为CD34的补充诊断免疫标志物，用于确诊DFSP并更好地评估残留/复发性肿瘤成分。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/791e97a10f96/cancers-13-00252-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/a08afe672f6d/cancers-13-00252-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/ae60dbfa6cbb/cancers-13-00252-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/81cfec567d0f/cancers-13-00252-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/3d411cdb43fe/cancers-13-00252-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/791e97a10f96/cancers-13-00252-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/a08afe672f6d/cancers-13-00252-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/ae60dbfa6cbb/cancers-13-00252-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/81cfec567d0f/cancers-13-00252-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/3d411cdb43fe/cancers-13-00252-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c485/7826654/791e97a10f96/cancers-13-00252-g005.jpg

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