Morbeck Diogo, Tregnago Aline C, Baiocchi Glauco, Sacomani Carlos, Peresi Patricia M, Osório Cynthia T, Schutz Luciana, Bezerra Stephania M, de Brot Louise, Cunha Isabela W
Department of Pathology, AC Camargo Cancer Center, São Paulo, Brazil.
Department of Gynecology, AC Camargo Cancer Center, São Paulo, Brazil.
Histopathology. 2017 Feb;70(3):435-441. doi: 10.1111/his.13086. Epub 2016 Nov 21.
GATA3 has been reported as a specific urothelial marker among organs in the pelvic region, and has been classified as highly sensitive and specific for urothelial and breast carcinomas. Our aim was to verify GATA3 expression in extramammary Paget disease, and to determine whether it can be use to differentiate primary vulvar Paget disease from pagetoid urothelial intraepithelial neoplasia (PUIN). We also analysed HER2 protein expression and HER2 gene amplification and their roles as prognostic factors in extramammary Paget disease.
We analysed GATA3 and HER2 expression in 11 primary vulvar Paget disease cases and two PUIN cases. All cases showed nuclear expression of GATA3. Of 13 cases, five were equivocal for HER2 expression (score 2+) and one was positive (3+). Fluorescence in-situ hybridization results showed amplification in two of these six cases. Both HER2-amplified cases were invasive.
GATA3 was positive in all extramammary Paget disease cases tested (13 cases), and it has no value for differentiating between primary and secondary vulvar Paget disease from the urological tract. HER2 amplification might confer an aggressive and invasive pattern in primary vulvar Paget disease, as both amplified cases showed an invasive pattern.
GATA3已被报道为盆腔区域器官中一种特异性的尿路上皮标志物，并且已被归类为对尿路上皮癌和乳腺癌具有高度敏感性和特异性。我们的目的是验证GATA3在乳腺外佩吉特病中的表达，并确定其是否可用于区分原发性外阴佩吉特病和佩吉特样尿路上皮内瘤变（PUIN）。我们还分析了HER2蛋白表达和HER2基因扩增及其在乳腺外佩吉特病中作为预后因素的作用。
我们分析了11例原发性外阴佩吉特病病例和2例PUIN病例中的GATA3和HER2表达。所有病例均显示GATA3核表达。在13例病例中，5例HER2表达不明确（评分2+），1例阳性（3+）。荧光原位杂交结果显示这6例中有2例扩增。这2例HER2扩增病例均为浸润性。
在所有检测的乳腺外佩吉特病病例（13例）中GATA3均为阳性，并且它对于区分来自泌尿道的原发性和继发性外阴佩吉特病没有价值。HER2扩增可能在原发性外阴佩吉特病中呈现侵袭性模式，因为2例扩增病例均显示为浸润性模式。
