1260
May occur in the nipple in conjunction with an invasive cancer mass, with an underlying ductal carcinoma in situ or without any underlying malignancy.
Paget’s cells thought to be derived from underlying mammary cancer or from the intraepidermal transformation of multipotential cells in the epidermal basal layer or from the terminal portion of the lactiferous duct at the junction with the epidermis.
Incidence has decreased between 1988 an 2002 for disease associated with underlying malignancy but the incidence of Paget’s disease alone has been stable.
Size of underlying tumors and nodal status are prognostic factor associated with survival.
Frequently misdiagnosed as eczema as it presents with a rash affecting the nipple areola complex.
Is seen in 1-3% of new breast-cancer cases, and typically found in postmenopausal women 50-60 years old.
Can occur in women of other ages and in men.
Produces a firmness of the underlying tissue rather than being a mere skin abnormalitiy.
Typically presents with an erythematous, scaling lesions of the nipple with burning, pain or itching at the site.
Flattening retraction of the nipple, spreading of the lesion to the areola, and bloody discharge indicates more advanced disease.
Bilateral involvement is extremely rare.
In 85-80% of cases the nipple ulceration is accompanied by an underlying ductal carcinoma in situ or invasive breast cancer.
Associated breast cancers maybe multifocal in nature.
In the remaining cases, Paget’s disease is restricted restricted to skin without associating malignancies and is called pure of Paget’s disease.
Pathogenesis is not well understood: two theories 1)epidermotropic holds Paget cells develop in the underlying breast malignancy and migraine via lactiferous ducts into the breast epidermis and 2) and the transformation theory suggests that epidermal keratinocytes become malignant and can transform into Paget cells.
No specific risk factors are known.
Workup includes bilateral mammography, ultrasonography and if studies are inconclusive, an MRI study.
Biopsy confirmation is required, with biopsy of the nipple lesion and any underlying breast abnormalities located by imaging studies.
Frequently HER2 positive and ER and PR negative.
TREATMENT
Options determined by the disease process.
If there is no multicentric disease and the underlying breast cancer is relatively close to the nipple-areola complex the patient may be a candidate for breast conserving surgery.
Neoadjuvant chemotherapy may allow some patients to undergo breast conserving therapy.
In patients with underlying malignancy or multicentric disease, mastectomy is the standard treatment.
In a review of 1642 cases mastectomy was not associated with a better survival than central lumpectomy.
Patients treated with mastectomy undergo sentinel lymph node biopsy.
Most patients receive whole breast irradiation, if breast conserving surgery is performed.
In patients with pure Paget disease do not receive post operative surgery.
PROGNOSIS
Prognosis depends on the stage and type of underlying disease.
SEER data: patients with Paget’s disease and underlying invasive breast cancer have a 15 year disease specific survival rate of 61%, whereas the rate is 94% for women with Paget disease with in situ carcinoma, and 88% for patients with pure Paget disease.
Tumor size and nodal status are major determinants of disease specific survival, as for all breast cancers.