What is Paget's Disease of the Nipple?

Paget disease of the nipple is manifested as scabs, shell-like erosions, or a type of fecal matter, which often looks benign, causing the patient to ignore and delay the diagnosis by one year or more. A positive diagnosis is made by a nipple biopsy, but a nipple cell smear is usually sufficient, and more than half of patients have a tangible mass at diagnosis. This potential cancer can be invasive or carcinoma in situ. The standard treatment is exactly the same as other forms of breast cancer. The prognosis depends on whether it is infiltrated, the size of the tumor, and the presence or absence of histological lymph nodes. Very few patients succeed with limited resection of the nipple and resection of some surrounding normal tissue. The disease usually occurs in middle-aged and older women, with an average age of 40 to 60 years, and it is rare in those under 40 years of age. Only a few cases can be male, and most often occur after the application of estrogen for prostate cancer.

Breast paget disease

Overview of breast paget disease

Breast paget disease description

This disease, also known as breast eczema-like cancer, is a special type of cancerous disease that mostly occurs in female breasts and can also occur in male breasts. It is mainly breast cancer or apocrine sweat gland cancer that spreads to the nipple and the surrounding epidermis. .

Causes of breast paget disease

It is currently believed that the disease originates near the opening of the mammary duct, which is intraductal carcinoma of the mammary duct and early stage carcinoma in situ. This intraductal cancer can invade the mammary glands or the parietal sweat gland epithelium inward, and invade the epidermis outward, forming epidermal lesions. Therefore, early tumor cells are in the duct, not in the epidermis, and later tumor cells break through the tube wall and enter the breast connective Within the organization. In a few cases, the lesions are located in deeper mammary ducts or glands. It can be seen that breast cancer coexists with this disease. Occasionally, the parietal sweat glands and epidermis originated in the nipple skin.

Pathophysiology of breast paget disease

Pathogenesis of breast paget disease

Breast paget disease pathological changes

In the early stage, the epidermal hypertrophy of the epidermis is accompanied by hyperkeratosis or incomplete keratosis, the epidermis is prolonged, and in the later stage, the epidermis becomes thinner, often with epidermal defects and even ulcers. The pathological changes of this disease are characterized by the appearance of Paget cells in the epidermis, especially in the lower part of the spinous layer. This cell is very different from epidermal cells, which is 1 to 2 times larger than normal keratinocytes, round, and free of cell spinous processes and intercellular bridges. , The cytoplasm is rich and lightly stained, such as vacuole-like. Nuclei are large, round or oval, deeply stained, and the nuclear membrane is clear. It is usually one or more cores. Visible mitosis. It can exist alone in the epidermis, or it can be nested together. It even invades all layers of the epidermis and squeezes the epidermal cells into a network. The basal cells are squeezed between the basement membrane band and Paget cells in a flat band shape. The so-called Paget-like phenomenon. Paget cells generally do not invade the dermis directly, but can spread along sweat ducts, sweat glands, hair follicles, and sebaceous glands. There is often moderate chronic inflammatory infiltration in the dermis. Intraductal cancer can be seen in the mammary ducts under the nipple. Cancer cells are similar to Paget cells. They involve the nipple and surrounding epidermis, and the mammary ducts, and even the mammary glands, penetrate the basement membrane and invade surrounding connective tissue, and become invasive cancer. Danger of spread and transfer.

Paget cells stained differently with histochemical staining. PAS staining was mostly positive and resistant to amylase. Weakly positive for Asin blue at pH 2.5. Melanin is occasionally seen but negative for dopa. Immunohistochemically stained Paget cells are often positive for epithelial membrane antigen and carcinoembryonic antigen, and can express C-erbB-2 oncoprotein and P53 anti-oncogene protein and estrogen receptor (ER) and progesterone receptor (PR) ).

Breast paget disease symptoms signs

It usually occurs in unilateral nipples, areola, and its surroundings. It has an eczema-like appearance, with clear red patches, mostly exudative crusts on the surface, gray-blue or gray-white keratotic desquamation, and cracks, Erosion or granulation tissue, bright red, often exudate. There is mild infiltration without noticeable itching. The skin lesions gradually expanded to the surrounding area, and the course of the disease was slow. After months or years, the lesions affected the breasts and chest. The edge of the lesion was slightly raised, with obvious infiltration, and scattered spots on the periphery. When the late damage spreads to the deep, the nipple begins to sink in, is damaged or even falls off, or ulcers occur. See also bloody nipple discharge. Half of the patients are accompanied by breast cancer and breast lumps, and metastasis is often found in advanced local lymph nodes.

Breast paget disease diagnostic test

This disease is a cancerous disease, so early diagnosis is very important. It should be distinguished from nipple eczema. If patients over 50 years of age have unilateral skin lesions, clear boundaries, basal infiltration, nipple discharge, or even nipple depression, the disease progresses slowly and relapses after temporary improvement. If the symptomatic treatment fails, this disease should be considered. The presence of Paget cells in the epidermis on a biopsy is very important for the diagnosis of this disease.

In the differential diagnosis, it should be mainly distinguished from eczema, Bowen's disease, and superficial malignant melanoma. At this time, pathological biopsy and histochemical and immunohistochemical staining are often required to confirm it.