Tran Mai N, Harvey Jennifer A
Radiation Oncology, Princess Alexandra Hospital Raymond Terrace, Brisbane, AUS.
Radiation Oncology, Princess Alexandra Hospital, Brisbane, AUS.
Cureus. 2020 Dec 8;12(12):e11966. doi: 10.7759/cureus.11966.
Extramammary Paget's disease (EMPD) is an uncommon entity, and secondary EMPD is even rarer. To our knowledge, this report involves one of the only three cases in the literature to date regarding the use of neoadjuvant radiation therapy in the treatment of secondary EMPD. A 65-year-old woman's EMPD had become more widespread over the years to involve buttocks, perineum, anus, vulva, and vagina. Given the knowledge of potential secondary EMPD, suspicious perianal lesions were biopsied. Histology and immunohistochemistry staining confirmed adenocarcinoma. Our patient was treated with neoadjuvant radiation therapy, along with concurrent chemotherapy. This was followed by pelvic exenteration, which confirmed a complete response from the neoadjuvant treatment. We discuss her presentation, investigations, and treatment regimen in detail. In addition, we review the treatment of secondary EMPD as reported in previously published literature.
乳腺外佩吉特病（EMPD）是一种罕见的疾病，继发性EMPD更为罕见。据我们所知，本报告涉及迄今为止文献中仅有的三例使用新辅助放疗治疗继发性EMPD的病例之一。一名65岁女性的EMPD多年来扩散至臀部、会阴、肛门、外阴和阴道。鉴于存在潜在继发性EMPD的可能，对可疑的肛周病变进行了活检。组织学和免疫组化染色证实为腺癌。我们的患者接受了新辅助放疗及同步化疗。随后进行了盆腔脏器清除术，证实新辅助治疗取得了完全缓解。我们详细讨论了她的临床表现、检查及治疗方案。此外，我们还回顾了既往发表文献中报道的继发性EMPD的治疗情况。
