Pap smears of patients with extramammary Paget's disease of the vulva

Department of Pathology, University of California Irvine Medical Center, Orange, California 92868, USA.
Diagnostic Cytopathology (Impact Factor: 1.52). 06/2005; 32(6):353-7. DOI: 10.1002/dc.20260
Source: PubMed

ABSTRACT Extramammary Paget's disease (EMPD) of the vulva is a rare entity. The diagnosis is almost always made on biopsy. Tumor cells are seen rarely in Papanicolaou (Pap) smears. We encountered three cases of EMPD that were detected in Pap smears. One patient had vulvar and vaginal involvement and the abnormal cells seen in the vaginal smear initially were interpreted as high-grade squamous intraepithelial lesion. Retrospective review showed scattered single atypical cells with enlarged hyperchromatic nuclei, coarse chromatin, inconspicuous nucleoli, high nuclear/cytoplasmic (N:C) ratio, and scanty basophilic cytoplasm. Rare signet ring cells and cells within cells were present. In the other two patients who had cervical involvement, the correct diagnosis was made on Pap smears. The slides showed both single and cohesive sheets of glandular cells with enlarged round to oval nuclei, coarse chromatin, prominent nucleoli, and abundant basophilic cytoplasm containing prominent vacuoles with signet ring-cell appearance. Cells within cells were abundant. EMPD has distinct cytomorphological features. Although infrequently encountered, EMPD can be diagnosed on Pap smears with adequate clinical history.

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    ABSTRACT: Introduction. 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. Case presentation. 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). Conclusion. 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.
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