Mammary Paget’s Disease of the nipple as a sign of local recurrence two decades following properly treated breast cancer

Purpose: Mammary Paget’s Disease (MPD) of the nipple-areolar complex (NAC) is a rare disorder of the breast associated with an underlying carcinoma affecting 1-4% of breast cancer patients. The etiology of MPD is thought to arise from a malignant conversion of pluripotent keratinocyte stem cells or cells of apocrine gland ducts; that is supported by structural similarity between Paget and Toker cells – epithelial cells found in the NAC of 10% of women. MPD presents as eczematous patches of scaly tissue surrounding the NAC associated with tingling and pruritis. Here we describe a 74-year-old patient with local recurrence of breast cancer >20 years following treatment.

Methodology: The patient initially presented with a right breast stage IA (cT1cN0M0) invasive ductal carcinoma involving right upper inner quadrant in year 2000. She was treated with partial mastectomy (PM) and sentinel lymph node biopsy (SLNB) followed by adjuvant whole breast radiation therapy and 2.5 years of adjuvant tamoxifen. She remained disease free until year 2022 when she presented with waxing and waning four-month history of skin irritation involving the right nipple. Exam demonstrated a small, 1 centimeter (cm), crusty erythematous rash. Initial trial of antibiotics did not improve symptoms. She underwent a punch biopsy which demonstrated MPD. Breast MRI showed a right NAC enhancement with a 0.5 cm mass at 12:00 o’clock position. Biopsy of the central mass demonstrated a grade 2 invasive ductal carcinoma, ER/PR+ without HER2 amplification. She underwent right mastectomy with SLNB with final pathology demonstrating stage IA disease (pT1apN0[sn]).

Results: This case highlights a unique clinical sign for breast cancer recurrence despite a long period of remission. Several factors are associated with a high risk of late recurrence including ER+ tumors, diagnosis before the age of 40, treatment involving breast conserving surgery, 4 or more positive lymph nodes, and a T2 or higher tumor stage. It is challenging to know if the new breast cancer is recurrent or new primary, although management is similar. For patients with underlying malignancy, the rate of SLNB was 17% warranting nodal evaluation. MPD local recurrence has been shown to be common in patients who previously underwent nipple sparing mastectomy. Small, single institution, series found that patients with underlying MPD had worse survival compared to those without MPD.

Conclusions: Unfortunately, no high quality, prospective, data exist to guide management for patients with MPD versus non-MPD recurrence; general approach includes SLNB followed by mastectomy; an alternative approach to mastectomy in women who recur a decade or two after initial PM and adjuvant radiation who are averse to mastectomy would be to repeat PM followed by partial breast radiotherapy. This case emphasizes the importance of early cancer surveillance to prevent delays in intervention.

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