In the clinic, we encountered some patients who found that the nipple areola became eczema-like and bought ointment for treatment by themselves without going to the hospital for formal treatment. Later, they found that the affected area was getting stronger and stronger before they went to the hospital for treatment and biopsy, only to find out that it was breast Paget’s disease.
Paget’s disease of the breast is a special type of breast cancer whose characteristic clinical manifestations are eczema-like changes such as itching, erosion, rupture, oozing, crusting, flaking and painful skin of the nipple areola, so it is also known as eczema-like carcinoma of the breast, which may or may not be accompanied by a lump in the breast. In 1874, Paget first reported 15 cases of eczema-like changes in the nipple areola, all with ipsilateral breast cancer, so this particular type of breast cancer was named Paget’s disease of the breast, accounting for 0.7% to 4.3% of breast cancers in the same period. The high incidence of Paget’s disease in the breast is between 50 and 54 years of age; the majority of cases are unilateral, while bilateral cases are rare. The pathology is characterized by the presence of Paget’s cells in the epidermis of the nipple, which appear round or oval under the microscope and are two to three times larger than the epithelial cells in the same layer, making them relatively large malignant cells. The prognosis of patients with Paget’s disease of the breast is good for those with simple nipple-areola lesions or those with intraductal carcinoma only; patients with breast lumps and pathologically confirmed invasive carcinoma of the lumps have a similar or slightly worse prognosis than patients with generalized breast cancer.
There are two explanations for the histogenesis of Paget’s disease of the breast, either originating from the ductal epithelium within the large ducts of the breast or from the flattened epithelium of the nipple in situ. The origin of Paget’s disease in the large ducts is based on the fact that the majority of patients with Paget’s disease in the breast have an intramammary mass and 90% of the pathological findings of these masses are invasive carcinoma of the breast, and even if the mass is not found on physical examination of the breast, postoperative pathology may reveal intraductal carcinoma. Also the expression of molecular biological factors in Paget’s cells is similar to that of breast cancer in the parenchyma of the breast. The origin of the papillary epithelium is thought to be based on the fact that although most cases of Paget’s disease of the breast are associated with intramammary breast cancer, there are still some patients who present with simple papillary lesions only, and in some cases of patients with intramammary breast cancer, the pathology suggests a discontinuity with the papillary lesion. In addition, ultrastructural observation of Paget’s cells revealed the possibility of in situ formation of Paget’s cells from flattened epithelial cells of the nipple.
Clinical presentation.
The clinical manifestation of Paget’s disease of the breast is eczema-like changes in the nipple areola area, with a progressive course with unilateral onset in most cases. The first abnormal sensation in the nipple area, manifested as itchy or mild burning pain in the nipple, followed by redness of the skin at the nipple areola, mild erosion, often with yellow-brown or gray scaly scabs attached to the surface, the lesion area skin rough, thickened and hardened, and clearly demarcated from the surrounding. Later, the affected nipple may become sunken or eroded and extend to the areola. It may be accompanied by nipple discharge. A mass can be palpated in the breast in most cases of Paget’s disease, and ipsilateral axillary lymph node enlargement may also be present in long-standing cases.
Examination.
1. Scrape and/or print cytology of the nipple areola lesion to look for Paget’s cells. Scraping cytology is the scraping of the lesion for smears. If the lesion is covered with scab or necrotic tissue, it should be removed first and then the exfoliated cells from the area should be taken for examination after fresh tissue is exposed. Patients with nipple discharge can be examined by cytological smear of nipple discharge. Since the above tests are less difficult to diagnose, they should be performed in hospitals with technical conditions.
Surgical biopsy, i.e. wedge-shaped excision of diseased tissue at the nipple and areola, including sufficient epithelium and breast ducts, for pathological histological examination, is the most effective diagnostic method.
3, breast imaging (mammography, ultrasound). Since the proportion of breast Paget’s disease with parenchymal carcinoma is >90%, and the percentage of patients with palpable lumps in the breast is >50%, and imaging examination can not only understand the lesions in the nipple areola area, but also show the signs of deep lesions and the whole breast and regional lymph nodes, which is not a bad auxiliary examination method.
Diagnosis.
1, history and signs: according to statistics: breast Paget’s disease from the patient’s symptoms to the diagnosis of about six months to two years, the longest surprisingly more than 20 years. The course of the disease is a gradual process. The disease is mainly centered on the nipple and gradually expands to the areola. In some cases, the disease improves locally with treatment and crusting, but soon ulcerates again, making it highly recurrent and incurable. Paget’s disease of the breast has characteristic signs: abnormal sensation in the nipple area, itching and burning pain, followed by redness, swelling and erosion, then ulceration and crusting, and after removing the scab, a red granulation surface and a small amount of exudate, and so on, eventually leading to the destruction of the nipple and areola, and the lesion begins to develop around the skin of the breast.
2, surgical biopsy, pathological histological examination, scraping, cytological examination of the print, is the “gold standard” for the diagnosis of breast Paget’s disease.
3, the incidence of breast Paget’s disease is low, the course of the disease is long, missed misdiagnosis cases are common. The eczema-like lesions on the nipple areola of breast Paget’s disease should be distinguished from eczema of the breast with special attention. Eczema of the breast is an inflammation of the breast skin caused by non-pathogenic agents. The cause is complex and is generally considered to be related to a metamorphic reaction, which may be linked to genetic factors. Nervousness and strain are also causative factors. Eczema of the breast is common in lactating women and often occurs on both sides of the breast at the same time. The lesion area is soft, with indistinct borders, no nipple deformation, and no masses in the breast. In contrast, Paget’s disease of the breast often occurs unilaterally, the lesion area is hard and has clear borders with the surrounding area, the nipple may become sunken or disappear if the disease is prolonged, and a lump may be palpable in the breast. The differential diagnosis between Paget’s disease of the breast and eczema of the breast is not difficult by biopsy (biopsy).
Treatment.
Surgery is the treatment of choice for Paget’s disease of the breast. If a lump can be palpated in the breast and it is confirmed to be invasive breast cancer intraoperatively, the treatment plan should be the same as that for breast cancer. Modified radical mastectomy (mastectomy plus axillary lymph node dissection) is feasible, followed by appropriate adjuvant therapy according to the pathology report, i.e. chemotherapy, radiotherapy, endocrine therapy, targeted therapy, etc. For patients with Paget’s disease whose clinical examination is limited to the nipple areola (no lesions outside the nipple areola area are detected by mammography and ultrasound), breast-conserving surgery is feasible, i.e., extended excision of the lesion, removing the nipple areola and its deep tissue, together with at least 2 cm of surrounding breast tissue, requiring negative margins and postoperative adjuvant radiation therapy. If the margins are positive, an extended excision or total mastectomy must be performed. Immediate (stage I) breast reconstruction can be performed depending on the patient’s needs. In principle, if the lesion is limited to the nipple areola and there is no mass in the breast parenchyma, or if there is only ductal carcinoma in situ, the axillary lymph nodes may not be cleared, but in practice it is difficult to control, because even if no enlarged lymph nodes are palpated in the axilla on clinical examination and no axillary lymph node metastasis is shown on imaging, the pathology report after modified radical surgery still shows axillary lymph node metastasis. Therefore, there is a discrepancy between the clinical examination alone and the postoperative pathological test results. Therefore, it is recommended to perform axillary lymph node dissection even after breast-conserving surgery. Postoperative adjuvant therapy should be followed as prescribed by the doctor.
Prevention.
Paget’s disease of the breast is a special type of breast cancer, the cause of which is not fully understood, so there is no definite method of prevention. From the analysis of epidemiological survey, the prevention of breast cancer can be considered in the following aspects.
1.Establishing a good lifestyle, adjusting the rhythm of life and keeping a relaxed mood.
2.Adhere to physical exercise, actively participate in social activities, avoid and reduce stressful factors, and keep a calm mind.
3.Adopt good eating habits.
4.Actively treat breast diseases.
5.Do not use exogenous estrogen indiscriminately.
6.Do not drink alcohol in excess for a long time.
Paget’s disease of the breast is a gradual and chronic process, first appearing abnormal sensation of the nipple, then involving the areola. Therefore, patients with itchy and painful nipples, especially nipple lesions that have not been treated as dermatological for more than two weeks, should be vigilant and see a mammographer for further diagnosis. It is recommended that women should learn some scientific knowledge about breast diseases, master breast self-examination methods, develop the habit of regular breast self-examination, and actively participate in breast cancer screening.