J Breast Cancer.  2010 Jun;13(2):227-230. 10.4048/jbc.2010.13.2.227.

Ectopic Extramammary Paget's Disease of the Breast Skin: A Case Report

• Hwang KT
• Kim H
• Chung JK
• Jung IM
• Heo SC
• Ahn YJ
• Ahn HS
• Chang MS
• Lee JH
• Noh DY

• KMID: 2286550
• DOI: http://doi.org/10.4048/jbc.2010.13.2.227

Abstract

Whereas extramammary Paget's disease commonly occurs in the apocrine gland rich skin areas, ectopic extramammary Paget's disease develops in the skin areas that are devoid of apocrine glands. We experienced the case of a 34 year-old female patient who had a skin lesion in the upper outer quadrant of the right breast for 5 years and that lesion was diagnosed as Paget's disease according to the punch biopsy. There was no other underlying malignancy, and so wide excision was performed. The final pathologic diagnosis was Paget's disease confined to the epidermis and the size of the tumor was 3.0x1.1 cm. Positive staining for cytokeratin 7, epithelial membrane antigen and negative staining for S-100 protein and HMB-45 was observed on the immunohistochemical tests. We report here on an extremely unusual case of ectopic extramammary Paget's disease of the breast skin, and we include a review of the relevant literature.

Keyword

Breast; Paget Disease Extramammary

MeSH Terms

Apocrine Glands
Biopsy
Breast
Epidermis
Female
Humans
Keratin-7
Mucin-1
Negative Staining
Paget Disease, Extramammary
S100 Proteins
Skin
Keratin-7
Mucin-1
S100 Proteins

Figure

• 

  Figure 1 Gross appearance of the breast skin lesion. A dark red skin lesion is observed in the upper outer quadrant of the right breast. The surface of the lesion is scaly and the margin is irregular but well defined. No discharge from the skin lesion is observed. There is no skin lesion in the nipple and areola.

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  Figure 2 Specimen of excised skin lesion. The size of the lesion is about 3.0×1.2 cm and each margin is painted with various colored ink to indicate the orientation of each direction. There is no elevation or depression of the lesion and no evidence of invasion to subcutaneous fat layer by manual examination.

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  Figure 3 Microscopic findings. The epidermis is infiltrated by large pleomorphic cells (i.e., Paget's cells) with abundant clear-staining cytoplasm which may be vacuolated in H&E stain, ×100 (A) and ×200 (B). These tumor cells show positive staining results for cytokeratin 7 (C) and negative staining results for S-100 protein (D) in immunohistochemistry staining, ×100.

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