GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15

Abstract

BackgroundGATA-binding protein 3 (GATA3) has been identified as a sensitive marker for breast carcinoma but its sensitivity in primary genital extramammary Paget diseases (EMPDs) has not been well studied.MethodsHere we investigated immunohistochemical expression of GATA3 in 72 primary genital EMPDs (35 from female, 37 from male; 45 with intraepithelial disease only, 26 with both intraepithelial disease and invasive adenocarcinoma including 14 also metastasis, 1 with metastatic adenocarcinoma only for study). We also compared GATA3 to gross cystic disease fluid protein 15 (GCDFP15) for their sensitivity.ResultsPositive GATA3 staining was seen in all 71 (100%) intraepithelial diseases, 25/26 (96%; female 10/10, male 15/16) invasive adenocarcinomas and 14/15 (93%; female 3/3, male 11/12) metastatic adenocarcinomas, respectively. Positive GCDFP15 staining was seen in 46/71 (65%; female 28/34 or 82%, male 18/37 or 49%) intraepithelial diseases, 20/26 (77%; female 9/10, male 11/16) invasive adenocarcinomas, and 12/15 (80%; female 2/3, male 10/12) metastatic adenocarcinomas, respectively (GATA3 versus GCDFP15: p < 0.01 for both intraepithelial disease and invasive adenocarcinoma, p = 0.28 for metastatic adenocarcinoma). In positive-stained cases, GATA3 stained more tumor cells than GCDFP15 (79% versus 25% for intraepithelial disease, 71% vs 34% for invasive adenocarcinoma, 73% vs 50% for metastatic adenocarcinoma, p < 0.01 for all 3 components).ConclusionsOur findings indicate that GATA3 is a very sensitive marker for primary genital EMPDs and is more sensitive than GCDFP15.

Perianal Paget’s disease: a clinicopathological and immunohistochemical study of 13 cases

“…Our results suggest that the presence of CDX2+/GCDFP-15-PPD should prompt a careful search for primary adenocarcinoma in the lower gastrointestinal tract as the underlying adenocarcinoma determines the outcome of the patient. GATA3 is a very sensitive marker for primary genital and vulvar extramammary Paget's disease [26,27]. It is also positive in primary PPD, although may not be used to differentiate from secondary PPD.…”

Stop routine screening for associated malignancies in cutaneous noninvasive vulvar Paget disease?

“…An example is GATA3, which is reported to be sensitive, and also a potential pitfall, in recognizing VPD secondary to urothelial malignancies. 24,25 Acknowledging this limitation, we assessed the risk of not screening for malignancy in those patients with a cutaneous IHC profile. Based on our data, refraining from additional screening for malignancies where IHC analysis shows a cutaneous profile would have missed five patients over 15 years in the Netherlands, a country of around 17 million inhabitants.…”

“…There are no studies assessing the sensitivity and specificity of IHC analysis in differentiating the different subtypes of VPD, and the reliability of several other IHC stains can be discussed. An example is GATA3, which is reported to be sensitive, and also a potential pitfall, in recognizing VPD secondary to urothelial malignancies …”

GATA3-Positive Adnexal Adenocarcinoma: Report of a Confusing Case with a Potential Pitfall of Leading to a Misdiagnosis of Urothelial Carcinoma and a Review of Published Work

“…Because the classification and the GATA3 sensitivity in MTAEDs are not well established, further study of a larger number of cases is needed. GCDFP15 is reported to be of cutaneous or mammary origin 11,12 . In the present case, the diagnosis of GATA3-positive adnexal adenocarcinoma was also confirmed by the positive staining of GCDFP15.…”