未经证实:乳腺外Paget病(EMPD)是一种罕见的疾病,其特征是外阴非鳞状细胞癌细胞上皮内累及皮肤,会阴,还有阴囊.二级EMPD甚至更罕见,约占病例的25%(Delport,2012)[1]。手术(Mohs/广泛局部切除术)是原发性非侵入性疾病的标准治疗方法,但继发性侵入性EMPD的管理定义较少,因为目前尚无治疗指南.
方法:一位76岁的女士有1年的肛周瘙痒性皮疹病史,对多种外用药物均难治。2020年6月,活检证实了EMPD的诊断,分期成像发现她的右腹股沟淋巴结有转移性疾病。在多学科团队(MDT)会议上讨论后,她进行了化学放疗,然后进行了全身化疗,因为她患有广泛的肛周疾病,会使手术变得非常病态。她每月进行3次间隔成像以评估对治疗的反应。2021年6月,患者有完整的临床反应,并正在进行6个月的监测。
UNASSIGNED:EMPD极为罕见,目前仍在浏览继发性EMPD的治疗方案。具体来说,目前尚无随机对照试验(RCT)比较放疗和手术之间的继发性EMPD结局以及III期疾病结局.该病例报告记录了受MDT共识支配的治疗方法,并进行了1年的随访。病例报告符合SCARE2020标准(Agha等人。,2020)[2]。
结论:患者的治疗效果良好,然而,需要持续的监测。
UNASSIGNED: Extra mammary Paget\'s disease (EMPD) is a rare disease characterised by intraepithelial involvement of the skin by non-squamous carcinoma cells in the vulva, perineum, and scrotum. Secondary EMPD is even rarer and accounts for roughly 25% of cases (Delport, 2012) [1]. Surgery (Mohs/wide local excision) is the standard treatment for primary non-invasive disease, but management of secondary invasive EMPD is less defined as there are no current treatment guidelines.
METHODS: A 76-year-old lady had a 1-year history of a pruritic perianal rash that was refractory to multiple topical agents. A biopsy confirmed a diagnosis of EMPD in June 2020 and on staging imaging was found to have metastatic disease in her right inguinal lymph node. Upon discussion at a multi-disciplinary team (MDT) meeting, she proceeded chemo radiation therapy followed by systemic chemotherapy as she had such extensive perianal disease that would make surgery quite morbid. She had interval imaging 3 monthly to assess response to treatment. In June 2021, the patient had a complete clinical response and has ongoing 6 monthly surveillance.
UNASSIGNED: EMPD is extremely rare and currently treatment options for secondary EMPD are still being navigated. Specifically, there are no current randomised control trials (RCTs) that compare outcomes in secondary EMPD between radiation therapy and surgery and in the context of stage III disease. This
case report documents the treatment approach governed by MDT consensus and has a 1 year follow up. The
case report has been reported in line with the SCARE 2020 criteria (Agha et al., 2020) [2].
CONCLUSIONS: The patient has had a good outcome with treatment, however ongoing surveillance is required.