Paget disease of the nipple: a multifocal manifestation of higher-risk disease.

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.

Breast carcinoma in women age 25 years or less.

BACKGROUND: Although several studies have examined breast carcinoma in young women aged < or = 35 years at diagnosis, there are only occasional cases reported in very young women aged < or = 25 years, and, to the authors' knowledge, no series are available. The presentation, tumor biology, behavior, and outcome of breast carcinoma in very young women are not known, and the rarity of breast malignancy within this age group could lead to diagnostic delays. METHODS: The tumor characteristics and survival of 15 women aged < or = 25 years at the time of diagnosis, have been reviewed and compared with women aged 26-35 years under the care of Guy's Hospital's Breast Unit during the same period of time. Where appropriate, the two groups were individually matched for tumor size (clinical measurement) and histologic grade. RESULTS: Fifteen cases were examined, with a median follow-up of 108 months and a median age of 24 years. The median duration of symptoms was 4 weeks, and the median tumor size was 20 mm. Two patients had ductal carcinoma in situ (DCIS) only, while the other 13 patients had invasive carcinomas, none of which were Grade I. A mastectomy was performed on 8 out of 15 patients (53%). Axillary nodal metastases were present in 4 out of 12 patients (33%). Of the 13 cases of invasive disease, 9 out of 13 patients (69%) experienced recurrence and died of breast carcinoma. Median disease free survival for patients with invasive disease was 86 months. There was no difference in overall survival between the patients aged < or = 25 years and those aged 26-35, but taken together young women < or = 35 had a worse prognosis than women between 36 and 65, due to a higher incidence of high grade and estrogen receptor negative tumors. CONCLUSIONS: The current study suggests that among young women with breast carcinoma there is no difference in prognosis between the very young and the young. Despite two thirds of patients being node negative, the high mortality rate indicates a need for an optimal selection of adjuvant therapy among these cases.