Cervicovaginal cytologic features in patients with Vulvar Paget disease

Justin L Burk1, Suzanne Crumley1, Xin Duan1

  • 1Department of Pathology and Immunology, Washington University, St. Louis, Missouri.

Abstract

Insights

Extramammary Paget disease (EMPD) can involve the cervix or vagina, as seen in 15% of Pap tests from patients with known EMPD. Recognizing specific cytologic features is key for accurate diagnosis of this rare condition.

Area of Science:

  • Gynecologic Pathology
  • Cytopathology
  • Dermatopathology

Background:

  • Extramammary Paget disease (EMPD) is a rare intraepithelial neoplasm primarily affecting the vulva or perineal skin.
  • Cervical or vaginal involvement by EMPD is infrequent, with limited reports of Paget disease identified in cervical cytology (Pap tests).

Purpose of the Study:

  • To determine the frequency of cervical/vaginal involvement in patients with known EMPD using Pap tests.
  • To characterize the cytologic features indicative of EMPD in Pap test samples.

Main Methods:

  • Retrospective review of Pap tests from patients diagnosed with vulvar or vaginal EMPD over a 10-year period.
  • Identification of cases through pathology LIS search and patient record review for concurrent or near-metachronous Pap tests.
  • Analysis of cytologic features suspicious for Paget disease involvement.

Main Results:

  • Out of 67 reviewed Pap tests from 40 patients with EMPD, 10 (15%) showed possible involvement.
  • Key suspicious cytologic features included hyperchromatic nuclei without visible nucleoli, increased nuclear-to-cytoplasmic ratios, and anisonucleosis.

Conclusions:

  • Cervicovaginal involvement by EMPD may be underdiagnosed on cytology.
  • A distinct set of cytomorphologic features associated with EMPD in Pap tests warrants further investigation.

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