Extensive Extramammary Paget's Disease With Underlying Perianal Adenocarcinoma: The Role of Neoadjuvant Radiation Treatment

Overview

Journal Cureus

Specialty Biomedical Engineering

Date 2021 Jan 11

PMID 33425542

Citations 3

Authors

Mai N Tran

Jennifer A Harvey

Affiliations

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Abstract

Extramammary Paget's disease (EMPD) is an uncommon entity, and secondary EMPD is even rarer. To our knowledge, this report involves one of the only three cases in the literature to date regarding the use of neoadjuvant radiation therapy in the treatment of secondary EMPD. A 65-year-old woman's EMPD had become more widespread over the years to involve buttocks, perineum, anus, vulva, and vagina. Given the knowledge of potential secondary EMPD, suspicious perianal lesions were biopsied. Histology and immunohistochemistry staining confirmed adenocarcinoma. Our patient was treated with neoadjuvant radiation therapy, along with concurrent chemotherapy. This was followed by pelvic exenteration, which confirmed a complete response from the neoadjuvant treatment. We discuss her presentation, investigations, and treatment regimen in detail. In addition, we review the treatment of secondary EMPD as reported in previously published literature.

Citing Articles

The impact of radiotherapy on disease control in vulvar extramammary Paget's disease: a retrospective study from a single institution.

Seol A, Kim S, Song Y Front Oncol. 2025; 15:1520528.

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Perianal Pagets disease complicated with lung adenocarcinoma and anal canal carcinoma: A case report and literature review.

Zhou L, Yang M, Wang T, Zhang J Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2023; 48(8):1274-1280.

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Rare Anal Canal Cancer with Secondary Extramammary Paget's Disease (Pagetoid Spread) Complicated by Squamous Cell Carcinoma of the Skin.

Kusuhara T, Ito T, Matsuoka H, Chohno T, Zozumi M, Ohnishi T Case Rep Surg. 2021; 2021:9944886.

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Perianal Paget Disease: Different Entities With the Same Name.

Santos M, Soares F, Presa-Fernandes J, Silva D Cureus. 2021; 13(5):e15161.

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