Apikotoa Sharie, Stein Joel
St John of God Hospital, Midland, 1 Clayton Street, Midland 6056, Western Australia, Australia.
St John of God Hospital, Midland, 1 Clayton Street, Midland 6056, Western Australia, Australia.
Int J Surg Case Rep. 2022 Feb;91:106796. doi: 10.1016/j.ijscr.2022.106796. Epub 2022 Jan 24.
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.
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.
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].
The patient has had a good outcome with treatment, however ongoing surveillance is required.
乳腺外佩吉特病（EMPD）是一种罕见疾病，其特征为外阴、会阴和阴囊皮肤的上皮内出现非鳞状癌细胞浸润。继发性EMPD更为罕见，约占病例的25%（德尔波特，2012年）[1]。手术（莫氏手术/广泛局部切除）是原发性非侵袭性疾病的标准治疗方法，但继发性侵袭性EMPD的治疗尚不明确，因为目前尚无治疗指南。
一位76岁女性有1年肛周瘙痒性皮疹病史，多种外用药物治疗无效。2020年6月活检确诊为EMPD，分期影像学检查发现其右侧腹股沟淋巴结有转移性疾病。在多学科团队（MDT）会议讨论后，由于其肛周疾病范围广泛，手术风险较大，她接受了化疗放疗，随后进行全身化疗。她每3个月进行一次间隔成像以评估治疗反应。2021年6月，患者临床完全缓解，目前每6个月进行一次监测。
EMPD极为罕见，目前继发性EMPD的治疗选择仍在探索中。具体而言，目前尚无随机对照试验（RCT）比较放疗和手术在继发性EMPD以及III期疾病情况下的疗效。本病例报告记录了由MDT共识指导的治疗方法，并进行了1年的随访。该病例报告已按照SCARE 2020标准（阿加等人，2020年）[2]进行报告。
患者治疗效果良好，但仍需持续监测。
