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Pathophysiology Summary-Paget Disease Research Paper

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Pathophysiology Extramammary Paget disease (EMPD) arises as a primary cutaneous adenocarcinoma in most cases. The epidermis becomes infiltrated with neoplastic cells showing glandular differentiation. Tumor cells may originate from apocrine gland ducts or from keratinocytic stem cells. Approximately 25% of the cases of EMPD (range, 9-32%) are associated with an underlying in situ or invasive neoplasm. In all patients, the neoplasm most likely to be associated with EMPD is an adnexal apocrine carcinoma. This associated neoplasm probably represents infiltration of the deeper adnexa by epidermal Paget cells. Other malignancies besides cutaneous adnexal carcinoma that may be associated with EMPD include carcinomas of the Bartholin glands, urethra, bladder, vagina, cervix, endometrium, and prostate. …show more content…

For instance, genital disease is associated with carcinoma in about 4-7% of patients. Perianal disease is associated with underlying colorectal carcinoma in 25-35% of cases. Rare cases of EMPD associated with tumors arising in distant organs without direct epithelial connection to the affected epidermis have been reported. Roy et al reported PD in a retrorectal dermoid cyst. [1] No clear evidence supports a causal link between these distant tumors and cutaneous

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