Department of Dermatology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Curr Treat Options Oncol. 2022 Jun;23(6):818-830. doi: 10.1007/s11864-021-00923-3. Epub 2022 Apr 4.
Extramammary Paget's disease (EMPD) is a rare neoplastic disease affecting areas rich in apocrine glands in the elderly. EMPD clinically resembles a benign inflammatory skin disease, and ill-defined tumor borders can lead to misdiagnosis and incomplete excision. Several prognostic factors have been reported, including nodule formation, tumor thickness, tumor invasion, lymphovascular invasion, and a perianal location, which are characteristic of primary tumors. EMPD typically presents as an in situ tumor spreading horizontally within the epidermis and then invading into the dermis as it transitions to a vertical growth phase. For this reason, tumor thickness, rather than tumor size, is correlated with patient prognosis. The best treatment for resectable EMPD is complete surgical removal of the tumor. EMPD sometimes has unclear tumor borders, and it can unexpectedly spread beyond its clinical boundaries. Surgical resection in such cases is often associated with tumor-positive margins, which can result in recurrence. However, surgical excision with wide margins may deteriorate patients' organ functions and quality of life. Mohs micrographic surgery may be ideal for controlling the surgical margins and minimizing the sacrifice of normal tissue, but this technique is not always feasible because of constraints associated with the medical environment. No standard treatment for unresectable or metastatic EMPD has been established. Although conventional chemotherapy has been used as the first-line treatment, it frequently causes adverse events, and consequently, targeted therapy will become more valuable in the near future.
乳房外帕哲病（EMPD）是一种罕见的肿瘤性疾病，好发于老年人富含大汗腺的部位。EMPD 临床上类似于良性炎症性皮肤病，且肿瘤边界模糊可导致误诊和不完全切除。已有多种预后因素被报道，包括结节形成、肿瘤厚度、肿瘤浸润、脉管浸润和肛周位置，这些都是原发性肿瘤的特征。EMPD 通常表现为原位肿瘤在表皮内水平扩散，然后在向垂直生长阶段过渡时侵入真皮。因此，肿瘤厚度而非肿瘤大小与患者预后相关。可切除 EMPD 的最佳治疗方法是完全切除肿瘤。EMPD 有时肿瘤边界不清晰，可能会出乎意料地超出其临床边界。在这种情况下，手术切除常伴有肿瘤阳性切缘，从而导致复发。然而，广泛切除边缘的手术可能会恶化患者的器官功能和生活质量。Mohs 显微外科手术可能是控制手术边缘和最小化正常组织牺牲的理想选择，但由于医疗环境的限制，该技术并非总是可行。对于不可切除或转移性 EMPD 尚无标准治疗方法。虽然常规化疗已被用作一线治疗，但它经常引起不良反应，因此，在不久的将来，靶向治疗将变得更加有价值。
