Molinie V, Paniel B J, Lessana-Leibowitch M, Moyal-Barracco M, Pelisse M, Escande J P
Service central Jacques Delarue d'Anatomie et de Cytologie Pathologiques, Hôtel Dieu, Paris.
Ann Dermatol Venereol. 1993;120(8):522-7.
Thirty six patients with Paget's disease of the vulva were reviewed. The median age of the patients at diagnosis was 67 years (range: 45-91 years). One patient had a history of previous mammary adenocarcinoma. Screening for malignancy revealed two colonic tumours. Two patients with negative screening at presentation developed, 12 and 18 months respectively after vulvectomy, an ovarian carcinoma stage IIc and a cervical and urethral adenocarcinoma. All patients were treated by surgery based on extent of the disease. The operations performed included total vulvectomy (n = 11), partial vulvectomy (n = 14) and wide local excision (n = 4). Out of the 36 patients, 29 were available to follow-up. The median follow-up period was 74 months (range 2-204 months). Three patients died of metastatic disease due to vulval adenocarcinoma and breast carcinoma, or of liver metastases from an unknown adenocarcinoma. Eighteen of the 29 patients followed up remained free of disease. Five out of the 16 patients with positive margins recurred, as did 5 out of 9 patients with negative margins. Treatment of Paget's disease of the vulva is surgical. In order to prevent recurrence, some authors have proposed surgical excision extending beyond the visible clinical lesions with intraoperative frozen sections. The data we recorded show that free margins do not seem to correlate with recurrence, so that large excision beyond the clinical lesion is not useful.
对36例外阴佩吉特病患者进行了回顾性研究。患者确诊时的中位年龄为67岁（范围：45 - 91岁）。1例患者有既往乳腺腺癌病史。恶性肿瘤筛查发现2例结肠肿瘤。2例初诊时筛查阴性的患者分别在 vulvectomy（此处可能有误，推测是外阴切除术，原文拼写不规范）后12个月和18个月，发生了IIc期卵巢癌以及宫颈和尿道腺癌。所有患者均根据疾病范围接受手术治疗。所进行的手术包括全外阴切除术（n = 11）、部分外阴切除术（n = 14）和广泛局部切除术（n = 4）。36例患者中，29例可进行随访。中位随访期为74个月（范围2 - 204个月）。3例患者死于外阴腺癌和乳腺癌的转移性疾病，或死于不明腺癌的肝转移。29例接受随访的患者中有18例无疾病复发。16例切缘阳性的患者中有5例复发，9例切缘阴性的患者中也有5例复发。外阴佩吉特病的治疗方法是手术。为了预防复发，一些作者建议手术切除范围超出可见的临床病变，并进行术中冰冻切片检查。我们记录的数据表明，切缘阴性似乎与复发无关，因此超出临床病变的大范围切除并无益处。
