Paget’s disease of the breast is often misdiagnosed. We report on a 72-year old patient with a history of 2.5 years without any malignant findings, followed by the identification of a bilateral Paget’s disease with bilateral breast cancers. This case underlines how important histological examinations even in unusual clinical pictures are.
Paget’s disease (PD) of the breast can be a diagnostic challenge. It might take years until the diagnosis. If the skin changes are intended to be benign but do not respond to topical therapy, a biopsy has to be performed to exclude malignancies. Almost all cases are single sided. We observed one of the rare cases of bilateral PD.
A 72-year-old woman (para 1) was seen with erythematous and eczematous patches that developed simultaneously on both nipples and had been present for 2.5 years (Figures
Clinical picture at time of first presentation and histological stains ((a) right breast, (b) cytokeratin 7 stain, (c) HE stain, and (d) left breast).
After 2.5 years she was referred to dermatology, where we biopsied both nipples. The histopathology showed epidermal cells with hyperchromatic and polymorphic nuclei, intraepithelial gland cells (Figure
Because of an induration of the left mamma and the incidence of underlying carcinomas, the patient was evaluated again by gynecologists who decided to operate on both breasts. They identified a bifocal invasive ductal carcinoma and an intermediate grade ductal carcinoma in situ (DCIS) of the left breast and a low-grade DCIS of the right central breast. Sentinel lymph nodes were not involved. Following surgery, the patient received chemotherapy with 6 cycles FEC (5-fluorouracil, epirubicin, and cyclophosphamide), trastuzumab, because of positive Her-2 status, radiotherapy, and tamoxifen. At 1.5-year follow-up being maintained on tamoxifen, she showed no relapse.
Between 1 and 4% of all breast cancers are Paget’s diseases [
The disease appears in three forms: (1) associated with an underlying ductal carcinoma in situ (DCIS), (2) associated with an invasive carcinoma, or (3) without any underlying malignancy [
For bilateral PD the limited data are controversial. Sahoo et al. [
It is still in discussion if the disease is the cause or consequence of an underlying malignancy. Most authors support the epidermotropism of malignant ductal gland cells into the epidermis. Some favor the migration of malignant keratinocytes from epidermis into deeper tissues, because in up to 50% of the cases no underlying tumors can be found [
Multiparous patients seem to have a reduced risk of ductal, lobular, tubular, and mucinous breast cancers. By contrast, the risk of medullary breast cancers increases with the number of pregnancies [
The cause for the bilateral form of PD remains uncertain as the number of patients is limited and the reported patients differ in age, gender, and ethnicity (see Table
Summary of all available cases of bilateral Paget’s disease.
Age/gender | Associated cancer | Country | Author/reference |
---|---|---|---|
53/female | L: intraductal carcinoma | USA | Anderson 1979/[ |
Female | ? | Netherlands |
Knol and Voorhuis 1981/[ |
Female | ? | India |
Sinha and Prasad 1983/[ |
Male | ? | India | Nagar 1983/[ |
74/female | ? | Portugal | Fernandes et al. 1990/[ |
Female | ? | Greece | Markopoulos et al. 1997/[ |
53/female | R: LCIS of the nipple, DCIS + microinvasive ductal carcinoma |
USA | Sahoo et al. 2002/[ |
73/female | R: high-grade intraductal carcinoma |
Italy | Franceschini et al. 2005/[ |
74/male | R: infiltrative ductal carcinoma | Turkey | Ucar et al. 2008/[ |
45/female | None | China | Xie et al. 2012/[ |
72/female | R: low-grade DCIS |
Germany | Barth 2014 |
L: left breast; R: right breast; DCIS: ductal carcinoma in situ; LCIS: lobular carcinoma in situ.
The treatment options are mastectomy or breast-conserving strategies including nipple excision or central lumpectomy with a lymph node biopsy. There are reports of positive lymph nodes even without any underlying malignancies [
Although radiological diagnostic tools have improved over the years, each suspicious skin lesion of the breast must be biopsied in order to avoid the progression of a malignancy. Our patient’s history of 2.5 years without any findings underlines the importance of early histological examinations.
The author declares that there is no conflict of interests regarding the publication of this paper.