Paget disease of the nipple

Abstract

BACKGROUND The treatment of Paget disease by mastectomy has been challenged recently in favor of breast‐conserving techniques. A large series of patients treated with mastectomy has been reviewed to assess the feasibility of less radical surgery. METHODS The cases of 70 women with a clinical diagnosis of Paget disease were reviewed. The type, grade, receptor and node status, and the mammographic and pathologic extent of the underlying breast malignancy were determined. The survival of patients with invasive disease was compared with matched controls without Paget disease. RESULTS The underlying malignancy was invasive in 58% of cases. Despite the fact that only one third of women presented with a palpable mass, the malignancy was frequently extensive, being confined to the retroareolar region in only 25% of cases. The true extent of the disease was underestimated by mammography in 43% of cases. Of the patients with ductal in situ carcinoma, 96.5% had high‐grade carcinomas and 100% had invasive carcinomas of high cytonuclear grade. Overexpression of the c‐erb‐B2 oncogene was detectable in 83% of cases. Patients with Paget disease had a significantly worse survival than matched controls, but this difference was eliminated if they were also matched for c‐erb‐B2 status. CONCLUSIONS Paget disease is often associated with extensive underlying malignancy, which is difficult to assess accurately either clinically or mammographically. As a consequence, cone excision of the nipple would have resulted in incomplete excision in 75% of cases. The underlying disease is of high grade and is frequently c‐erb‐B2 positive with a resulting poor prognosis. Aggressive local and systemic treatment would seem to be merited. Cancer 2002;95:1–7. © 2002 American Cancer Society. DOI 10.1002/cncr.10638

Surgical treatment in Paget’s disease with invasive ductal carcinoma: an observational study based on SEER

“…Kothari et al 13. reported that patients with PD-IDC had a significantly lower survival (10-year OS 49%) than patients with IDC only (64%).…”

Paget′s disease of the breast

“…[1112] Approximately 50% of this patients present with an associated palpable mass in the breast. [34] In cases where mass is palpable, invasive carcinoma is likely to be found.…”

“…[13] The tumors which may or may not be palpable, located usually close to areola, tend to be central and are often multifocal. [11] Patients with periphery localized tumors also have been reported. In one study, Chaudary et al .…”

“…[14] Multifocality of the underlying carcinoma in Paget's disease has been reported to be between 42% and 63% in various studies. [1113] Lymph node enlargement is found more often in cases with palpable tumor. [13] Of note, Paget's disease of the breast may be asymptomatic and unsuspected clinically and may be diagnosed as a histologic finding by a pathologist on a mastectomy specimen.…”

An Unusual Case of Mammary Paget’s Disease Diagnosed Using Dynamic Contrast-Enhanced MRI

“…Mammography findings include skin thickening, malignant calcification, or masses at the level of the nipple, architectural distortion, and nipple retraction. However, the literature reports that the mammography can be negative in 22%–50% of patients [2, 5, 13, 14]. As underlying carcinoma is common even in women with a benign mammogram and no palpable mass, the breast US and MRI may be useful in detecting the lesion.…”