Sir James Paget first described Paget's disease of the breast (PD) in 1874. He reported a chronic eczematous disease of the nipple and areola skin in 15 women and the underlying intraductal carcinoma of the mammary gland.
Paget's disease of the breast (PD) is almost exclusively seen in women; involvement of the male breast is rarely reported and can be more aggressive. Patients with PD often present with chronic, eczematous rashes on the nipple and adjacent areolar skin. Proper recognition of this disorder is necessary to distinguish it from other benign skin conditions and to detect underlying breast cancer.
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Symptoms of Paget's Disease of the Breast
Patients with Paget's disease of the breast (PD) often have a history of relatively long-standing eczematous skin lesions or persistent dermatitis on the nipple and adjacent areas. Eczematous skin lesions are associated with various symptoms, including:
- Erythema
- Scaling
- Itching
- Burning sensation
- Ulceration
- Nipple discharge
- Bleeding
- A combination of the above symptoms
Early signs and symptoms of Paget's disease of the breast include:
- Lesions caused by scratching due to itching
- Resolution and recurrence of small vesicles within the skin lesion
Symptoms like pain, itching, and burning sensation prompt patients to seek medical attention. Scaly, erythematous, crusty, and thickened plaques on the nipple extend to the surrounding breast tissue.
Diagnosis of Paget's Disease of the Breast
Over 98% of patients have nipple changes associated with an underlying breast cancer (either the onset of breast cancer or invasive ductal carcinoma); two-thirds of patients have a palpable breast tumor.
Nipple biopsy from the scaling area can confirm the diagnosis.
Unilateral involvement is the rule; however, bilateral Paget's disease of the breast has occasionally been reported. Pigmented Paget's disease of the breast is seen in both men and women. These diseases can clinically and histopathologically mimic malignant melanoma and can also mimic melanoma under dermoscopic examination. Pigmented lesions of Paget's disease may contain an increased number of benign melanocytes, which could mislead the diagnosis and result in a false interpretation as malignant melanoma.
Ultrasound abnormalities in the nipple-areola complex may be noticeable.
Since this tumor can be multifocal and multicentric, breast MRI is recommended to assess the true extent of the disease.
Mammography
Radiographic changes seen in Paget's disease of the breast (PD) include:
- Subareolar microcalcifications (important for assessing and locating clinically hidden, non-palpable underlying breast cancer)
- Distortion of breast shape
- Thickening of the nipple and areola (reflecting edema)
- Nipple changes (in a minority of patients)
Positive findings on mammography are seen in about 50-70% of cases of biopsy-proven Paget's disease of the breast; guided biopsy based on mammographic findings is helpful.
In 94% of cases where biopsy-proven Paget's disease is the sole physical finding, underlying cancer is present. However, negative preoperative mammography findings do not reliably exclude underlying cancer. Statistical evidence suggests that magnetic resonance imaging (MRI) of the affected breast can detect Paget's disease in cases of negative mammography findings, facilitating treatment planning for patients diagnosed with Paget's disease.
Treatment of Paget's Disease of the Breast
Mastectomy (simple or modified with lymph node dissection) is appropriate treatment for patients with Paget's disease of the breast (PD) with palpable masses and underlying invasive breast carcinoma. Up to two-thirds of patients are reported to have axillary lymph node metastases. About 65% of those without palpable masses have non-invasive breast carcinoma (in situ carcinoma).
More limited surgery involves local excision of the nipple, wedge resection of the underlying breast, and a combination of radiation therapy. Few patients are treated with one or more conservative measures (e.g., nipple excision and wedge excision of the underlying breast, cone excision, radiation therapy). Breast-conserving treatment with lumpectomy and radiation is an effective option for patients with Paget's disease-ductal carcinoma in situ and Paget's disease-invasive ductal carcinoma compared to mastectomy.
Recurrence has been observed in patients treated with cone excision and elective tamoxifen therapy. Thus, cone excision alone is not sufficient for patients whose disease is confined to the nipple.
Wide local excision with axillary lymph node sampling is recommended regardless of the presence of a clinical mass.
Radiotherapy alone may not always control occult breast cancer but can be used for patients who refuse mastectomy or are medically unfit for surgery.
