Paget Disease of the Breast With Invasion From Nipple Skin Into the Dermis: An Unusual Type of Skin Invasion Not Associated With an Adverse Outcome

Abstract

Context.—Paget disease is an uncommon skin manifestation of breast cancer, associated with either invasive carcinoma or ductal carcinoma in situ in the underlying breast. In very rare cases, tumor cells within the epidermis invade through the basement membrane of the skin into the dermis. Objectives.—To identify a series of cases of Paget disease with direct dermal invasion and to investigate the clinicopathologic features and outcome. Design.—Cases were identified during a 6-year period from the files of 2 hospitals. The clinical histories, imaging studies, and pathology reports were reviewed. Results.—Seven patients were identified, 5 with microinvasion (<0.1 cm) and 2 with 0.2- or 0.3-cm invasive carcinomas in the dermis. No lymphovascular invasion was seen. Sentinel nodes were negative in 3 patients who underwent biopsy. Five patients were treated with breast conservation with radiation. Three patients were at high risk for breast cancer because of prior breast cancer, Li-Fraumeni syndrome, or radiation for Hodgkin disease. The latter 2 patients underwent bilateral mastectomies. Three patients received hormonal therapy and 1 oophorectomy. No patient received chemotherapy. At follow-ups ranging from 4 to 66 months (median, 20 months), there have been no recurrences. Conclusions.—Patients with direct dermal invasion from Paget disease had a favorable outcome during the available follow-up period. This type of dermal involvement must be distinguished from locally advanced invasive carcinomas with skin invasion classified as T4b in the American Joint Cancer Commission staging system, as cancers with other types of skin invasion are associated with a poor prognosis.

Recurrence of breast carcinoma as Paget disease of the skin at a prior core needle biopsy site: Case report and review of the literature

“…Fortunately, the carcinoma was detected while still in situ. Invasion can occur from Paget disease and the delay in diagnosis could have resulted in development of invasive carcinoma and a poorer prognosis [16,17] .…”

Invasive Paget Disease of the Nipple

“…To our knowledge, 17 cases of IPDN have been documented (Table 1). 1-5 No clinical or detailed pathological information is ascertainable for the 2 cases illustrated by Rosen. 1 Among the other 15 cases, not a single case was node positive, none exceeded 3 mm in extent of invasion, and no case had any evidence of disease after initial treatment in 7 to 108 months of follow-up.…”

“…Invasive PDN (IPDN) is a relatively recently recognized disease entity, characterized by invasive carcinoma that is localized to the nipple and is associated with PDN and either intraductal and/or invasive carcinoma of the underlying breast. In all, 17 cases of IPDN have been reported, 1-5 with no case reported as node positive. Although the vast majority of PDN are of the classical type, it is important to be able to identify the 2 less-common types of PDN—that is, the secondary and invasive types—mainly to enable appropriate clinical management and also to accurately stage the disease.…”

Paget's disease of the male breast

“…This type of dermal involvement must be distinguished from locally advanced invasive carcinomas with skin invasion classified as T4b in the American Joint Cancer Commission staging system, as cancers with other types of skin invasion are associated with a poor prognosis. 21 Although there seems to be no significant difference in the tumor histology in male and female, the former appears to have a poor prognosis. The five-year survival rate in males is 20 to 30% and in females 30 to 50 %.…”