Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2024 Aug 23;16(8):e67589.
doi: 10.7759/cureus.67589. eCollection 2024 Aug.

Simultaneous Occurrence of Two Ectopic Extramammary Paget Disease Lesions: A Case Report

Kenji Yorita  1 , Norihiro Hokimoto  2 , Keiko Sakamoto  3 , Satoka Yoshii  3 , Keisuke Kashiwagi  3
Affiliations
Case Reports

Simultaneous Occurrence of Two Ectopic Extramammary Paget Disease Lesions: A Case Report

Kenji Yorita et al. Cureus. .

Abstract

Paget disease of the breast is a malignant tumor that occurs primarily on the skin of the nipple, whereas extramammary Paget disease occurs on the skin in regions other than the breast, such as the pubis, perianal area, and axilla. Paget disease can also arise outside these areas; this is referred to as ectopic extramammary Paget disease. In this study, we present a case of a Japanese woman in her 70s who experienced simultaneous occurrence of two ectopic extramammary Paget disease lesions on the skin of the anterior chest and left buttock. Both lesions, exceeding 5 cm in diameter, were surgically excised, and the histopathological examination led to a diagnosis of ectopic extramammary Paget disease without invasion. To the best of our knowledge, this is the third reported case of multiple simultaneous ectopic extramammary Paget disease. Clinically, diagnosis of a patient with ectopic extramammary Paget disease is challenging because of its unusual location; however, the disease may be considered a differential diagnosis for Bowen disease.

Keywords: ectopia; extramammary paget disease; pathology; skin; surgery.

PubMed Disclaimer

Conflict of interest statement

Human subjects: Consent was obtained or waived by all participants in this study. IRB of Japanese Red Cross Kochi Hospital issued approval None. The study design was approved by the appropriate ethics review board. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Gross images of ectopic extramammary Paget disease lesions on the left anterior chest (upper inner quadrant of the left breast) and the left buttock
Two reddish-brownish skin lesions are observed on the left side of the anterior chest (A, arrow) and on the upper medial side of the left buttock (B, arrow). The anterior chest skin lesion is located in the upper inner quadrant of the left breast (A). The border of these lesions is clear; however, a discontinuous area is observed at the edge of the anterior chest skin lesion (A, arrowhead). These skin lesions are at a distance away from the left nipple, left areola, and perianal area.
Figure 2
Figure 2. Pathological images of the ectopic extramammary Paget disease lesions on the left anterior chest (upper inner quadrant of the left breast) and the left buttock
Atypical cuboidal cells forming tubules are observed in the epidermis (A: hematoxylin and eosin-stained specimen). Immunohistochemically, these tumor cells are diffusely positive for cytokeratin 7 (CK7) (B), gross cystic disease fluid protein 15 (GCDFP15) (C), GATA-binding protein 3 (GATA3) (D), and human epidermal growth factor receptor 2 (HER2) (E) and negative for CK20 (F), p63 (G), and S100 (H). The scale bars are shown in the figure.
Figure 3
Figure 3. Pathological images of the ectopic extramammary Paget disease of the left buttock
Atypical cuboidal cells forming tubules or solid nests are observed in the epidermis (A: hematoxylin and eosin–stained specimen). Immunohistochemically, these tumor cells are diffusely positive for cytokeratin 7 (CK7) (B), gross cystic disease fluid protein 15 (GCDFP15) (C), GATA-binding protein 3 (GATA3) (D), and human epidermal growth factor receptor 2 (HER2) (E) and negative for CK20 (F), p63 (G), and S100 (H). The scale bars are shown in the figure.
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Mammary and extramammary Paget's disease. Kanitakis J. J Eur Acad Dermatol Venereol. 2007;21:581–590. - PubMed
    1. Patterson JW. Vol. 2021. Poland: Elsevier; 2021. Weedon's Skin Pathology, 5th ed; pp. 844–847.
    1. Calonje E, Damaskou V, Lazar AJ. McKee's pathology of the skin, 5th ed. Vol. 2. 2020: Elsevier; 2020. Cutaneous metastases and Paget disease of the skin; pp. 1539–1544.
    1. Definition, association with malignancy, biologic behavior, and treatment of ectopic extramammary Paget's disease: a review of the literature. Scarbrough CA, Vrable A, Carr DR. https://pubmed.ncbi.nlm.nih.gov/31531170/ J Clin Aesthet Dermatol. 2019;12:40–44. - PMC - PubMed
    1. Ectopic extramammary Paget's disease affecting the upper abdomen. Onishi Y, Ohara K. https://pubmed.ncbi.nlm.nih.gov/8736346/ Br J Dermatol. 1996;134:958–961. - PubMed

Publication types

LinkOut - more resources