Invasive Acantholytic Extramammary Paget's Disease of the Vulva With Mammary Carcinoma-Like Differentiation and MMR Loss: Report of an Unusual Case and Its Clinical Mimics.

Jessica Claus, Janina Pearce, Areta Bojko, Padmini Manrai, M Ruhul Quddus, Shivali Marketkar

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Article in Gynecologic oncology reports, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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6 authors.

Jessica ClausDepartment of Pathology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

Janina PearceProgram in Women's Oncology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

Areta BojkoProgram in Women's Oncology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

Padmini ManraiDepartment of Pathology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

M Ruhul QuddusDepartment of Pathology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

Shivali MarketkarDepartment of Pathology, Women and Infants Hospital, Warren Alpert Medical School, Brown University, Providence, RI, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Paget's disease (EMPD) of the vulva primarily affects postmenopausal women and can present significant challenges in early clinical detection. Conclusion: In the literature, acantholytic Paget's disease and acantholytic anaplastic Paget's disease have been used interchangeably. While anaplastic Paget's disease has been reported in the mammary and extramammary sites (scrotum, esophagus), involvement of the vulva has been reported only once in the literature. However, the current case is the first documented loss of MMR. Both cases presented with invasive disease ranging in depth from 3-5 mm, and positive lymph nodes emphasizing on the urgent need to further explore prognostic factors of this rare entity and to differentiate it from other acantholytic lesions, such as squamous cell carcinoma in situ and other acantholytic dermatoses.

Identifiers

PMID41561668

DOI10.1016/j.gore.2025.102007

PMCPMC12813102

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