乳腺外Paget病(EMPD)是一种经常复发的恶性肿瘤,具有转移潜力,在老年人的生殖器上表现出来,肛周,和腋窝皮肤.乳房外Paget病可以先于内部恶性肿瘤或与内部恶性肿瘤一起发生。
为EMPD成人提供护理建议。
使用MEDLINE对1990年1月至2019年9月18日有关EMPD的文献进行了系统回顾,Embase,WebofScience核心合集,和Cochrane图书馆。分析包括483项研究。对研究结果的多学科专家小组评估导致了EMPD临床护理建议的制定。
主要发现如下:(1)多次皮肤活检,包括任何结节区域的区域,对诊断至关重要。(2)应在基线进行适合年龄和解剖部位的恶性肿瘤筛查,以区分原发性和继发性EMPD。(3)不建议常规使用前哨淋巴结活检或淋巴结清扫术。(4)对于表皮内EMPD,根据患者和肿瘤特征,可以使用手术和非手术治疗,虽然治愈率可能优于手术方法。对于侵入性EMPD,有治愈意图的手术切除是首选。(5)不可切除的表皮内EMPD患者或医学上无法接受手术的患者可以接受非手术治疗,包括放射治疗,咪喹莫特,光动力疗法,二氧化碳激光治疗,或其他方式。(6)远处转移性疾病可用化疗或个体化靶向治疗。(7)建议至少在前5年内密切随访以监测复发。
EMPD的临床实践指南为推荐的诊断方法提供了指导。区分侵袭性和非侵袭性疾病,以及手术和非手术治疗的使用。前瞻性登记可以进一步改善我们对原发性和继发性EMPD疾病的自然史的理解,阐明高危肿瘤的特征,并确定卓越的管理方法。
Extramammary Paget disease (EMPD) is a frequently recurring malignant neoplasm with metastatic potential that presents in older adults on the genital, perianal, and axillary skin. Extramammary Paget disease can precede or occur along with internal malignant neoplasms.
To develop recommendations for the care of adults with EMPD.
A systematic review of the literature on EMPD from January 1990 to September 18, 2019, was conducted using MEDLINE, Embase, Web of Science Core Collection, and Cochrane Libraries. Analysis included 483 studies. A multidisciplinary expert panel evaluation of the findings led to the development of clinical care recommendations for EMPD.
The key findings were as follows: (1) Multiple skin biopsies, including those of any nodular areas, are critical for diagnosis. (2) Malignant neoplasm screening appropriate for age and anatomical site should be performed at baseline to distinguish between primary and secondary EMPD. (3) Routine use of sentinel lymph node biopsy or lymph node dissection is not recommended. (4) For intraepidermal EMPD, surgical and nonsurgical treatments may be used depending on patient and tumor characteristics, although cure rates may be superior with surgical approaches. For invasive EMPD, surgical resection with curative intent is preferred. (5) Patients with unresectable intraepidermal EMPD or patients who are medically unable to undergo surgery may receive nonsurgical treatments, including radiotherapy,
imiquimod, photodynamic therapy, carbon dioxide laser therapy, or other modalities. (6) Distant metastatic disease may be treated with chemotherapy or individualized targeted approaches. (7) Close follow-up to monitor for recurrence is recommended for at least the first 5 years.
Clinical practice
guidelines for EMPD provide guidance regarding recommended diagnostic approaches, differentiation between invasive and noninvasive disease, and use of surgical vs nonsurgical treatments. Prospective registries may further improve our understanding of the natural history of the disease in primary vs secondary EMPD, clarify features of high-risk tumors, and identify superior management approaches.