Paget’s disease of the breast is a form of breast cancer that occurs with a lesion of the nipple-areolar complex. Disease is manifested by itching, burning, nodular compaction and erosion in the periarticular area, discharge from the nipple. Diagnosis is based on clinical and cytological data, results of mammography, breast ultrasound, MRI. Treatment includes a mastectomy followed by radiation therapy, chemotherapy or hormone therapy.
Paget’s disease of the breast – eczema-like nipple cancer accounts for 0.5-5% of all cases of neoplastic breast changes in mammology. The disease was first described by J.Paget in 1874 as areola and nipple cancer with the phenomena of chronic eczema. Pathology occurs not only in women, but also in men, occurring mainly after 50-60 years. In men, Paget’s disease of the breast is diagnosed less often, but has a more aggressive course, which is associated with the smaller size of the mammary glands and easier penetration of cancer cells into the lymph nodes. Extra–mammary Paget cancer can develop in other areas where apocrine sweat glands are located: on the vulva and perineum in women; on the penis and scrotum – in men.
The etiology is unknown. Currently, mammology has several theories regarding the cause of the development of this disease. According to the epidermotropic theory, atypical cells (Paget cells) in breast cancer of deeper localization migrate through the milky ducts to the nipple, where they are implanted and grow. This theory is based on observations showing that Paget’s disease of the breast in 95% of cases is combined with other forms of breast cancer, more often with intracurrent carcinoma. The epidermis-infiltrating Paget tumor cells in nipple cancer originate from the epithelium of the milky ducts and are glandular cells.
According to the transforming theory, the development of Paget’s disease results from the spontaneous degeneration of the epithelial cells of the nipple and areola into malignant ones. This hypothesis is based on those cases in which breast cancer of a different localization is not detected. The family predisposition to the development of Paget’s disease of the breast in men, as well as its association with scleroderma, pemphigus, cervical cancer, is described. Among the provoking factors, injuries of the nipple, various carcinogenic effects are considered.
According to the variant of the clinical course, Paget’s disease is isolated, flowing:
- in the form of acute eczema (with fine-grained rash, wetness and ulceration of the nipple)
- in the form of chronic eczema (with the formation of crusts, when removed, a patch of wetness is formed)
- in psoriatic form (with the presence of small pink papules covered with dry peeling scales)
- in ulcerative form (with the formation of crater-like ulceration)
- in tumor form (with the presence of tumors in the thickness of the gland).
In this disease in 50% of cases, changes affect only the nipple-areolar complex. In 40% of patients, against the background of changes in the areola and nipple, a palpable tumor node is detected in the periarticular region. In 10% of cases, Paget’s disease is an accidental finding during cytological examination of a smear from the nipple in the presence of secretions from the ducts of the breast.
Early symptoms of Paget’s disease of the breast are non-specific, as a rule, do not cause concern and do not become a reason for contacting a mammologist. The disease begins with a slight redness, peeling, slight irritation of the skin of the nipple and the periarticular area. These phenomena may spontaneously disappear, which, however, does not indicate the disappearance of the disease. Temporary weakening of eczematous changes may occur when using corticosteroid ointments.
In the future, there is tingling, burning, itching, hypersensitivity, pain in the nipple. There may be serous hemorrhagic discharge from the nipple, soiling the underwear, changing the configuration of the nipple (flattening or retraction). The progression of the symptoms of Paget’s disease of the breast is accompanied by the development of stagnant infiltration and hyperemia in the area of the nipple and the periarticular circle, on the surface of which erosions, crusts, ulceration are formed. The removal of scales and crusts leads to the exposure of a wet, wet surface. More often, unilateral changes are observed in Paget’s disease, but the bilateral nature of the disease in both nipples is not excluded.
Eczematous changes can spread to the skin of the breast. The lesion area is usually slightly raised above the unaffected skin and clearly outlined. On palpation, nodular formations in the chest and an increase in regional lymph nodes are detected in half of the patients. In the later stages, due to the destruction of the nipple-areolar complex, abundant bloody discharge from the affected area occurs. In men, Pathology has similar manifestations – peeling, visible erythema, compaction, erosion, itching in the periarticular area, retracted nipple, maceration of the skin.
The complex of diagnostic studies includes consultation with a mammologist-oncologist, examination of the mammary glands, breast ultrasound, mammography, MRI, cytological examination of the smear, breast biopsy. When examining the breast, characteristic changes are found in the nipple area; with palpation, nodular tumor formations can be determined in the thickness of the tissues. Examination of the smear-print of the nipple being separated allows you to detect Paget cells.
The sensitivity of mammography in the diagnosis increases significantly in the presence of a tumor node in the gland; in the absence of the latter, the sensitivity of X-ray diagnostics does not exceed 50%. When analyzing mammograms, attention is paid to the shape of the nipple, the thickness of the skin of the nipple-areolar region, the presence of subareolar microcalcinates, the localization and size of the tumor node. To exclude tumor formations of another histological structure, mammography should be performed in three projections, supplemented with tomosynthesis.
In some cases, changes that are not detected during mammography can be visualized using ultrasound of the mammary glands; in addition, echography allows you to determine the state of the ducts and the nature of angiogenesis. Breast MRI is promising for detecting changes that have not yet manifested clinically, as well as differentiation of tumors of various types. Mammary gland scintigraphy is highly informative for detecting nipple lesions, since dividing intra-current Paget cells are able to actively accumulate the 99tTs radioisotope.
A biopsy of the nipple and areola, as well as a puncture biopsy of palpable formations, makes it possible to establish a histological diagnosis of disease at the preoperative stage. The diagnosis of Paget’s disease of the breast requires differentiation from eczema of the nipples, superficial basal cell carcinoma, melanoma, dermatitis, scabies, psoriasis, Bowen’s disease. In addition, it is necessary to distinguish Paget’s disease of the breast from herpes infection, fungal mycosis, tuberculosis and breast syphilis.
When detecting Paget’s disease of the breast, surgical tactics are indicated. Radical mastectomy is performed for invasive forms of cancer or widespread intracurrent carcinoma. In these cases, the mammary gland is removed along with the pectoral muscles, lymph nodes and fiber.
A simple mastectomy with the removal of gland tissue and small pectoral muscle is justified in non-invasive cancer growth. In the early stages, radical resection of the breast can be used, including the removal of the nipple, areola and part of the gland with a tumor located in it. In the future, patients undergo reconstructive mammoplasty.
In addition to surgery for Paget’s disease of the breast, remote gamma therapy, chemotherapy, and hormone therapy are prescribed. The objective of systemic treatment is to prevent recurrence of breast cancer and metastasis of Paget’s disease of the breast, especially in young patients.
The primary recognition does not always occur in a timely manner, and therefore adequate treatment may be delayed for many months.
Given the aggressiveness of tumor growth, the prognosis is difficult. Even after surgery, there is a high probability of relapse. The average life expectancy for Paget’s disease of the breast is about 3 years, with the presence of an infiltrative component and metastases – about 1 year.