PDF(Pubmed)
Abstract：
Anal adenocarcinoma combined with perianal Paget\'s disease (PPD) involving the vulva is rare, and there is no established standard treatment. We present the case of a 69-year-old woman with symptoms of intermittent hematochezia and perianal discomfort for 7 months. Upon examination, we discovered a plaque-like hard mass on the right posterior wall of the anal canal, which extended to encompass the anus and dentate line. The lesion skin also extended forward from the gluteal groove, involving the bilateral labial area. Colonoscopy revealed an extensive protruding lesion on the dentate line, which was confirmed as anal adenocarcinoma (mrT4N0M0). The presence of Paget\'s cells in perianal and vulvar skins led to the diagnosis of PPD. The strategy of neoadjuvant chemoradiotherapy (nCRT) followed by radical surgery was then made after multi-disciplinary discuss. The scope and extent of perianal and vulvar disease were significantly diminished after nCRT. The patient underwent laparoscopic abdominoperineal resection and vulvar lesion resection, confirming the diagnosis of anal adenocarcinoma (ypT2N0). No evidence of tumor cells was found in perianal and vulvar skin, indicating a complete response. The patient is regularly monitored without recurrence or metastasis.
摘要：
肛门腺癌合并肛周Paget病（PPD）累及外阴是罕见的，也没有既定的标准治疗。我们介绍了一名69岁女性，其间歇性便血和肛周不适症状持续7个月。经检查,我们在肛管的右后壁上发现了一个斑块状的硬块，延伸到包括肛门和齿状线。病变皮肤也从臀沟向前延伸，涉及双侧唇区。结肠镜检查显示齿状线上有广泛的突出病变，经证实为肛门腺癌（mrT4N0M0）。Paget细胞在肛周和外阴皮肤中的存在导致了PPD的诊断。经过多学科的讨论，制定了新辅助放化疗（nCRT）后根治性手术的策略。nCRT后，肛周和外阴疾病的范围和程度显着减弱。患者接受了腹腔镜腹会阴切除术和外阴病变切除术，确认肛门腺癌（ypT2N0）的诊断。在肛周和外阴皮肤中没有发现肿瘤细胞的证据，表示完整的响应。定期监测患者，无复发或转移。
