code of breast: XH3E21: extramammary: XH70F8
3.3.3 Paget’s Disease
Grading & Level of Importance: B
Rare disease. 3% of all breast cancers.
Uncommon intraepidermal adenocarcinoma, arising from intraductal carcinoma in the breast (mammary Paget’s disease) Can also arise from apocrine sweat glands or underlying carcinomas e.g. rectum, prostate (extramammary Paget’s disease).
Aetiology & Pathogenesis
Associated breast cancer is of the intraduct variety.
Signs & Symptoms
Circumscribed, erythematous, sometimes scaly, increasingly infiltrated plaque. Nipple (unilateral), axilla, groin or perianal. Caution: for chronic dermatitis involving one nipple -> rule out Paget’s disease.
Nipple, axilla, genitalia, lower abdomen, perianal.
Large clear intraepithelial tumour cells, PAS positive.
Slowly and gradually spreads.
Association with intraductal carcinoma (Paget’s disease) or adenocarcinoma (extramammary Paget’s disease).
Clinical features and histology.
Nipple dermatitis, perianal dermatitis, psoriasis.
Prevention & Therapy
Surgical excision adjusted to tumour size and underlying disease, multidisciplinary approach.
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