Inconsistent Immunostaining and Racial Difference in Extramammary Paget's Disease

乳房外柏哲德氏病之免疫染色與人種差異性研究

蔣富強(Fu-Chiang Chiang) ； 朱思穎(Szu-Ying Chu) ； 張雲亭(Yun-Ting Chang)

《中華皮膚科醫學雜誌》 27卷3期 (2009/09) Pp. 161-169

https://doi.org/10.29784/DS.200909.0003

摘要

背景：乳房外之柏哲德氏病(Paget's disease)為一少見之皮膚癌病，常發生於大汗腺豐富處，呈濕疹樣斑塊。依據癌細胞的來源，可區分該疾病為原發性或次發性。病理下可見非典型明亮細胞之表皮內浸潤。
目的：探討乳房外柏哲德氏病之病理與免疫染色特徵能否預測其臨床表現或是否伴隨內部腫瘤。
方法：我們回溯性地收集過去十年間22例新診斷的乳房外柏哲德氏病，所有病患皆為華人，歸納分析患者的病歷，並重新檢視其皮膚病理以及免疫染色下的發現。免疫染色試劑選用cytokeratin 7(CK7)、cytokeratin 20 (CK20)，以及gross cystic disease fluid protein-15(GCDFP-15)。
結果：沒有任何病患被診斷為次發性乳房外柏哲德氏病。所有的病灶標本裡，柏哲德氏細胞的CK7免疫染色皆呈陽性。原發性的案例裡，58%的病灶標本其柏哲德氏細胞呈現CK20(-)/GCDFP-15(+)的結果。結論：對乳房外柏哲德氏病的華人患者而言，常規性的安排侵入性檢查或許並非必要。CK7因具高敏感性，可以幫助診斷乳房外柏哲德氏病。以CK20與GCDFP-15的染色結果區分原發性或次發性並不可靠。

並列摘要

Background: Extramammary Paget's disease (EMPD), an uncommon neoplastic disorder, usually presents as an eczematous patch on the apocrine-rich area. It can be further classified into primary and secondary EMPD by its association with underlying malignancies. The histopathology shows a characteristic intraepidermal atypical clear cell infi ltrate.
Objective: To determine whether the histopathologic or immunohistochemical features of EMPD could be used to predict clinical behavior or association with underlying malignancies.
Methods: Twenty-two freshly-diagnosed EMPD cases were collected retrospectively in the past 10 years. All were Chinese and their medical records were reviewed. Histopathology of skin specimens were examined and immunohistochemical staining of cytokeratin 7 (CK7), cytokeratin 20 (CK20), and gross cystic disease fl uid protein-15 (GCDFP-15) were performed. Results: None of the cases were proven to be secondary EMPD. The Paget's cells from all cases were immunopositive for CK7. In 58% of primary EMPD cases, the Paget's cells had the immunophenotype CK20(-)/GCDFP-15(+).
Conclusion: In Chinese patients with EMPD, routine invasive surveys for associated malignancies might be unnecessary. CK7 is a suitable marker for the diagnosis of EMPD due to its high sensitivity. It is unreliable to distinguish between primary and secondary EMPD from their reactivity of CK20 and GCDFP-15.

並列關鍵字

Extramammary Paget's disease ； Cytokeratin 7 ； Cytokeratin 20 ； Gross cystic disease fluid protein-15