Background/Objectives: Although clinicopathologic correlation with integration of clinical and radiographic data is the gold standard in distinguishing primary extramammary Paget disease (EMPD) from secondary EMPD, immunoprofiling of EMPD tumors enables distinction between primary and secondary EMPD.Methods: We evaluated the immunoprofiles of previously published cases in the literature as well as 12 secondary EMPD cases from our archives in order to construct a diagnostic algorithm that enables the distinction between primary and secondary EMPD.Results: Immunoprofiles of 480 primary (published cases) and 132 secondary (120 published cases and 12 institutional cases) EMPD cases were compared. CK7, CK20, CDX2, GATA3, GCDFP15, TRPS1, and SATB2 expression was significantly different in primary EMPD versus colonic secondary EMPD (p< 0.001 for all except SATB2,p= 0.036). CK20, GCDFP15, TRPS1, p63 and uroplakin II/III expression was significantly different in primary EMPD versus urothelial secondary EMPD (p< 0.001). CK7, CDX2, SATB2, GATA3 and p63 expression was significantly different in colonic versus urothelial secondary EMPD. CK20, CDX2, and GCDFP15 expression was significantly different in colonic versus prostatic secondary EMPD. CK20 expression was significantly different in colonic versus prostatic secondary EMPD (p= 0.018). CK20, GCDFP15 and TRPS1 are helpful in the distinction of primary EMPD versus colonic and urothelial secondary EMPD (p< 0.001).Conclusions: We propose that the initial IHC panel should include TRPS1, CK7 and CK20. In TRPS1-negative cases, additional immunostains should be performed: CDX2 and SATB2 for colonic; p63, GATA3 and uroplakin II/III for urothelial; and PSA and NKX3.1 for prostatic secondary EMPD.
背景/目的：虽然结合临床与影像学数据的临床病理关联是区分原发性与继发性乳房外佩吉特病（EMPD）的金标准，但EMPD肿瘤的免疫谱分析能够有效鉴别原发性和继发性EMPD。 方法：我们评估了文献中已发表病例及本机构档案中12例继发性EMPD的免疫谱特征，旨在构建一套能够区分原发性和继发性EMPD的诊断算法。 结果：比较了480例原发性（已发表病例）和132例继发性（120例已发表病例及12例本机构病例）EMPD的免疫谱。CK7、CK20、CDX2、GATA3、GCDFP15、TRPS1及SATB2在原发性EMPD与结肠源性继发性EMPD中的表达存在显著差异（除SATB2的p=0.036外，其余指标p<0.001）。CK20、GCDFP15、TRPS1、p63及尿斑蛋白II/III在原发性EMPD与尿路上皮源性继发性EMPD中的表达具有显著差异（p<0.001）。CK7、CDX2、SATB2、GATA3和p63在结肠源性与尿路上皮源性继发性EMPD间的表达存在显著差异。CK20、CDX2和GCDFP15在结肠源性与前列腺源性继发性EMPD间的表达具有显著差异，其中CK20的表达差异尤为显著（p=0.018）。CK20、GCDFP15和TRPS1对区分原发性EMPD与结肠源性及尿路上皮源性继发性EMPD具有重要价值（p<0.001）。 结论：我们建议初始免疫组化检测组合应包括TRPS1、CK7和CK20。对于TRPS1阴性病例，应追加特异性免疫染色：结肠源性加做CDX2和SATB2；尿路上皮源性加做p63、GATA3和尿斑蛋白II/III；前列腺源性加做PSA和NKX3.1。
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