Review Question - QID 221299

Paget Disease of the Breast Prognosis Dependent upon presence of the underlying invasive ductal carcinoma or axillary node metastases QID: 221299 (M2.OMB.4693)

QID 221299 (Type "221299" in App Search)

A 48-year-old woman presents to her primary care doctor with a 1 month history of an itchy rash on her right breast. She has a long history of atopic dermatitis and has been consistently using over-the-counter 1% hydrocortisone cream without relief. She has also occasionally noticed pink discharge on her bra at the end of the day. Her medical history is significant for hypertension and hyperlipidemia, for which she takes lisinopril and atorvastatin. Her temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. The physician cannot palpate any breast masses or axillary lymphadenopathy bilaterally. The lesion on her right breast is shown in Figure A. Her left nipple and areola appear normal, without retractions or erythema. Which of the following diseases is most associated with this condition?

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Adenocarcinoma

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Inflammatory breast cancer

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Intraductal papilloma

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Phyllodes tumor

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Squamous cell carcinoma

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This patient with erythematous and pruritic nipple and areolar changes unresponsive to corticosteroids most likely has Paget disease of the breast. This condition is associated with underlying adenocarcinoma.

Paget disease of the breast is a chronic, eczematous rash of the nipple and areola. The skin changes seen with this condition are thought to be the result of malignant cell migration through the mammary ducts to the nipple surface. An underlying adenocarcinoma, most often ductal carcinoma in situ, is present in nearly 100% of cases. Patients will present with an erythematous, eczematous, potentially ulcerating rash along with pain, burning, itching, or bleeding. These lesions typically have no response to topical corticosteroids. Further workup includes mammography to locate an underlying malignancy, scrape cytology to detect malignant Paget cells, and biopsy of the lesion. Treatment of Paget disease of the breast depends on the treatment of the underlying breast cancer.

Schelfhout et al. reviewed the role of the HER2/NEU receptor in the spread of Paget cells through the nipple epidermis. They found that Paget cells express heregulin receptors as well as HER2/NEU receptors. They recommend further study of the role of chemotactic factors released by breast keratinocytes in the migration of malignant cells in Paget disease of the breast.

Figure/Illustration A is a clinical photograph showing eczematous changes of the nipple and areolar region (red circle). These changes are classically seen with Paget disease of the breast.

Incorrect Answers:
Answer 2: Inflammatory breast cancer is often a type of invasive ductal carcinoma. The malignant cells obstruct the lymphatic vessels, causing the characteristic inflammatory appearance. It will present as breast redness and pain with peau d’orange changes in the skin.

Answer 3: Intraductal papilloma is a benign epithelial tumor located in the lactiferous ducts of the breast. It will usually present as painless bloody nipple discharge in a woman under 50.

Answer 4: Phyllodes tumor is a rare, benign tumor known to have a leaf-like appearance on biopsy. The typical presentation is a breast mass that is rapidly growing, firm, mobile, and nontender.

Answer 5: Squamous cell carcinoma in situ (Bowen disease), in extremely rare cases, can present on the nipple as an erythematous lesion with hyperkeratosis, desquamation, and pruritus. A small percentage of patients will progress to invasive squamous cell carcinoma.

Bullet Summary:
Paget disease of the breast is associated with underlying adenocarcinoma.