Content area
Full Text
ABSTRACT
Mammary Paget's disease (PD) is a rare occurrence that is highly associated with breast ductal carcinoma in situ (DCIS) and invasive ductal carcinoma (IDC). PD is characterized by aggressive changes to the nipple and areolar regions. Due to the rarity of this disease, evidencebased recommendations for management of PD are limited. Currently, mastectomy alone and breast conserving surgery paired with adjuvant radiation therapy (RT) are considered equally effective in treating PD. In this case series, two women presented with similar cases of PD of the nipple, though only one had associated DCIS; both women received or will receive RT after breast conserving surgery. PD is rare, and diagnosis is often missed or prolonged. More research on the treatment and outcomes of PD is recommended.
Keywords: Mammary Paget's disease, radiotherapy, breast cancer, ductal carcinoma in situ
Introduction
Mammary Paget's disease (PD: also known as PD of the nipple) is a rare occurrence in breast cancer patients, associated with about 1 - 4% of all breast carcinomas (1). It presents more commonly in postmenopausal women aged 50-60 years, with a median age of 56 years (2).
Although this disease is uncommon, it does have a strong correlation with underlying breast cancer, as 92-100% of PD cases are associated with ductal carcinoma in situ (DCIS), invasive ductal carcinoma (IDC), or both (1,3,4).
PD involves the invasion of epidermal cells of the nipple and areola by malignant Paget's cells, often leading to excessive discolouration or pigmentation enclosed by an irregular border (1). The skin changes around the areola often cause discomfort and are generally described as thick, pigmented, moist, scaly and/ or crusty, with extreme cases of ulceration (5). PD is often detected by an erythematous appearance and can be mistaken for chronic eczema or a rash, however the skin irritation associated with PD is more aggressive and persistent than a rash and diagnosis requires a skin punch biopsy to sample epithelial cells (6). Unlike common breast carcinomas where skin erythema is not an indicator of disease, PD skin abnormalities are a primary feature of disease (1, 6). Currently, there are no characteristics or features that have been identified as increasing the risk of PD (1).
This series presents two cases of PD, both with persistent areolar...