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Paget's disease of the breast, nipple and areola has been reported as the presenting sign of breast cancer in 0.5% to 4.3% of all cases. It is extremely rare in the first three decades of life. The peak incidence is between 50 and 60 years of age, with a median of 56 years, 5 to 10 years higher than the overall peak incidence of invasive breast carcinoma. The mean duration of symptoms prior to presentation is 6.5 months (range 1 to 54), indicating an inappropriate delay in making the diagnosis Paget's Disease. One-third of the patients with Paget's disease overall are premenopausal, and some reports have indicated a slightly higher incidence of the disease.
About 40 case reports in the literature involve Paget's disease in the male breast. Classically, Paget's disease is described as a unilateral lesion. Bilateral lesions are exceptional, and there is only one case report of Paget's disease occurring in an accessory nipple. Inspection of the nipple and areola reveals a well-delineated, demarcated, slightly infiltrated, erythematous, exudative, or scaly area, which the patient often describes as burning or itching . The lesion frequently appears first on the nipple, subsequently spreads to the areola.
Any eczema or vesicular eruption on the nipple must be regarded as Paget's disease until proven otherwise. Persistent pain, soreness, or itching, despite the absence of a clear lesion, is suspicious. Mammography is important, particularly in light of the recent trend toward more conservative treatment of Paget's disease. In the absence of a palpable mass in the breast upon presentation, mammography is used to detect and locate subclinical underlying tumors, clusters of suspicious microcalcifications.
When the diagnosis of Paget's disease is suspected, confirmation must be promptly obtained with a full-thickness nipple biopsy. Exfoliative or scrape cytology with demonstration of Paget's cells.
The treatment of Paget's disease must take into account not only the etiologic concepts of the disease, but also the pathologic data behind it. The widespread acceptance of the epidermotropic theory.
Results from various early series in the literature demonstrated early on a clear difference in the natural history of patients who presented with a palpable mass in the breast as opposed to patients with nipple changes only. Patients with nipple changes only had 5- and 10-year survival rates of 90% to 100% following mastectomy, as opposed to 20% to 60% for patients with a palpable mass at 5 years.
The uniformly good prognosis for patients who present with Paget's disease of the nipple in the absence of a palpable tumor has led to some attempts at conservative management of this lesion. Lagios et al described five patients treated by excision of the affected nipple-areola complex.
Based on recent evidence that irradiation of the breast is effective in controlling DCIS, Fourquet et al, at the Institute Curie in Paris in 1987, treated 17 patients with Paget's disease of the nipple.
Despite the encouraging results noted in these reports, experience with nipple-areola excision and radiation therapy for Paget's disease of the nipple remains very limited. Furthermore, one must consider the natural history of the underlying carcinoma being treated, namely, DCIS.
As wide local excision followed by radiation therapy became a well-established modality in the treatment of invasive breast carcinoma, the logical next step was its application.
Whereas tamoxifen has been shown to decrease the incidence of ipsilateral as well as contralateral breast cancer after breast-conserving therapy for invasive carcinoma, its role in the treatment of DCIS.