Takahashi Kotaro, Kikuchi Akira, Horiuchi Ayano, Tamura Ryo, Haino Kazufumi, Yamaguchi Masayuki, Kawasaki Takashi
Department of Gynecology, Niigata Cancer Center Hospital, Niigata, Japan.
Department of Obstetrics and Gynecology, Nagaoka Red Cross Hospital, Niigata, Japan.
Am J Case Rep. 2021 Oct 22;22:e933655. doi: 10.12659/AJCR.933655.
BACKGROUND Vulvar extramammary Paget disease (EMPD) with abnormal cervical cytology is extremely rare. We encountered a case of secondary EMPD derived from urothelial carcinoma diagnosed after cytological examination for cervical cancer screening. We diagnosed the case promptly owing to suspicion based on the patient's medical history and vulvar appearance. We report the case and present a review of published cases of EMPD with abnormal cervical cytology. CASE REPORT A 77-year-old Japanese woman visited a hospital because cervical cancer screening raised the suspicion of adenocarcinoma. Findings of the cytological examinations of the cervix, endometrium, and urethral meatus corresponded to those of other malignant neoplasms of the Bethesda system. The patient had undergone total urethral cystectomy for urothelial carcinoma 5 years earlier. In our hospital, we found erythema extending from the urethral meatus to the vulva and performed a vulvar biopsy based on the suspicion of recurrence of the urothelial carcinoma. We diagnosed secondary EMPD derived from the urothelial carcinoma based on the findings of Paget cells in the epithelium and immunohistochemistry. CONCLUSIONS A review of all the reported cases of EMPD with abnormal cervical cytology shows that the frequency of primary lesions is high in primary EMPD and secondary EMPD derived from urothelial carcinoma. These cases demonstrated the difficulty of suspecting EMPD based on cervical cytology alone. It should be considered that the cells derived from vulvar EMPD can be observed in cervical cytology, particularly in patients with a history of primary EMPD or urothelial carcinoma and with vulvar symptoms.
伴有异常宫颈细胞学检查结果的外阴部乳腺外佩吉特病（EMPD）极为罕见。我们遇到了一例继发于尿路上皮癌的EMPD病例，该病例是在宫颈癌筛查的细胞学检查后确诊的。由于基于患者病史和外阴外观的怀疑，我们迅速诊断了该病例。我们报告此病例，并对已发表的伴有异常宫颈细胞学检查结果的EMPD病例进行综述。病例报告：一名77岁的日本女性因宫颈癌筛查怀疑为腺癌而就诊于一家医院。宫颈、子宫内膜和尿道口的细胞学检查结果与贝塞斯达系统的其他恶性肿瘤相符。该患者5年前因尿路上皮癌接受了全尿道膀胱切除术。在我们医院，我们发现从尿道口延伸至外阴的红斑，并基于对尿路上皮癌复发的怀疑进行了外阴活检。根据上皮中佩吉特细胞的发现和免疫组化结果，我们诊断为继发于尿路上皮癌的EMPD。结论：对所有报告的伴有异常宫颈细胞学检查结果的EMPD病例的综述表明，原发性EMPD和继发于尿路上皮癌的EMPD中原发灶的发生率较高。这些病例表明仅根据宫颈细胞学检查怀疑EMPD存在困难。应考虑到在外阴EMPD患者的宫颈细胞学检查中可观察到来源于外阴EMPD的细胞，尤其是有原发性EMPD或尿路上皮癌病史且伴有外阴症状的患者。
