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Extramammary Paget’s Disease

  • Chapter
Cutaneous Adnexal Neoplasms

Abstract

Extramammary Paget’s disease is more common in women than in men, and the vulva is the most common location.

Histopathologically, the characteristic cells of extramammary Paget’s disease show ample and pale cytoplasm and round and pleomorphic nuclei, which often shows prominent nucleoli. The cytoplasm shows a basophilic staining as well as a finely granular appearance due to abundant mucin. Sometimes, the cytoplasmic content of mucin displaces the nucleus to the periphery and then the cell acquires signet ring cell morphology.

Paget’s disease of the breast is an adenocarcinoma of the breast in which of neoplastic cells extend to the epidermis of the areola.

Carcinomas of the rectum, vagina, uterus, prostate, urethra, and bladder may extend by contiguity to the epidermis of the perianal or genital skin mimicking extramammary Paget’s disease.

Immunohistochemically neoplastic cells of extramammary Paget’s disease express CEA, human milk fat globule membrane antigen (HMFG), human breast cancer antigen MRB-1, GCDFP-15, Ca 15-3 and KA-93, erbB-4 and erbB-2, CK7, MUC1, and MUC5AC, but not CK20 and MUC2.

Extramammary Paget’s disease shows an indolent course, remaining confined to the epithelium of the epidermis and cutaneous adnexa for long periods of time and without metastasis.

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  1. Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain

    Luis Requena

  2. Department of Pathology, Wake Forest University School of Medicine, Winston Salem, North Carolina, USA

    Omar Sangüeza

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Requena, L., Sangüeza, O. (2017). Extramammary Paget’s Disease. In: Cutaneous Adnexal Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-45704-8_33

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