Clayton Erica F, Rubin Stephen C, Dumoff Kimberly L
Hospital of the University of Pennsylvania, Philadelphia, PA, USA
Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
Int J Surg Pathol. 2014 Jun;22(4):374-7. doi: 10.1177/1066896913498819. Epub 2013 Aug 5.
Paget's disease of the vulva is a rare malignancy primarily affecting Caucasian women in their seventh to eighth decades. Most patients experience an indolent disease course and undergo surgical excision for disease control. Although progression to invasive adenocarcinoma is rare, recurrence is common because of the difficulty of achieving negative surgical margins.
We report a case of a 73-year-old woman with a long-standing history of recurrent vulvar Paget's disease who presented with postmenopausal bleeding. Workup revealed extensive endocervical involvement by Paget's disease, resulting in Paget cells on endocervical curettage, as well as endometrial curettage (because of cervical contamination).
Extensive endocervical involvement by vulvar Paget's disease can occur despite multiple reexcisions and topical therapy. The presence of Paget cells on endometrial and endocervical curettings, particularly in patients without visible vulvar or cervical lesions, should raise suspicion of endocervical involvement and prompt further evaluation of disease extension.
外阴佩吉特病是一种罕见的恶性肿瘤，主要影响70至80岁的白种女性。大多数患者病程进展缓慢，通过手术切除来控制病情。尽管进展为浸润性腺癌的情况罕见，但由于难以实现手术切缘阴性，复发很常见。
我们报告一例73岁女性，有复发性外阴佩吉特病的长期病史，出现绝经后出血。检查发现宫颈广泛受佩吉特病累及，宫颈刮除物及子宫内膜刮除物（因宫颈污染）中均有佩吉特细胞。
尽管进行了多次再次切除和局部治疗，外阴佩吉特病仍可广泛累及宫颈。子宫内膜和宫颈刮除物中出现佩吉特细胞，尤其是在没有明显外阴或宫颈病变的患者中，应引起对宫颈受累的怀疑，并促使对疾病扩展进行进一步评估。
