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<p>Extramammary Paget’s disease (EMP) is a&#xa0;rare, slow-growing cutaneous adenocarcinoma with an incidence of 0.1–2.4 per 1,000,000 inhabitants. Histomorphologically, typical Paget cells can be identified and two pathogenetically and prognostically different forms are distinguished: the primary form, which is classified as carcinoma in situ of the apocrine sweat glands, and the secondary form, which is characterized as intraepithelial spread of associated apocrine-differentiated malignant tumors. EMP occurs predominantly in patients aged between 50&#xa0;and 80&#xa0;years, with European women and Asian men most frequently affected. EMP very often develops in the anogenital region and less frequently in the axillae. The diagnosis and treatment of EMP is challenging as the symptoms are often initially nonspecific and resemble chronic inflammatory dermatoses, which can lead to delays in diagnosis. Micrographically controlled excision is the preferred therapy for primary EMP; however, the recurrence rate remains high at up to 40%. Other treatment options include radiotherapy and the use of local therapeutic agents. In secondary EMP, the focus is on treating the underlying primary tumor; in the metastatic stage, the prognosis is unfavorable.</p>

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Extramammärer Morbus Paget

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月10日

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  • Hermann Kneitz,
  • Matthias Goebeler

摘要

Extramammary Paget’s disease (EMP) is a rare, slow-growing cutaneous adenocarcinoma with an incidence of 0.1–2.4 per 1,000,000 inhabitants. Histomorphologically, typical Paget cells can be identified and two pathogenetically and prognostically different forms are distinguished: the primary form, which is classified as carcinoma in situ of the apocrine sweat glands, and the secondary form, which is characterized as intraepithelial spread of associated apocrine-differentiated malignant tumors. EMP occurs predominantly in patients aged between 50 and 80 years, with European women and Asian men most frequently affected. EMP very often develops in the anogenital region and less frequently in the axillae. The diagnosis and treatment of EMP is challenging as the symptoms are often initially nonspecific and resemble chronic inflammatory dermatoses, which can lead to delays in diagnosis. Micrographically controlled excision is the preferred therapy for primary EMP; however, the recurrence rate remains high at up to 40%. Other treatment options include radiotherapy and the use of local therapeutic agents. In secondary EMP, the focus is on treating the underlying primary tumor; in the metastatic stage, the prognosis is unfavorable.