Paget ‘s Cancer

Paget ‘s Cancer
Paget’s cancer is one of the rare types of breast cancer that affects the nipple-areolar complex. It is characterized by manifestations in the form of nodular seals, itching, burning, peeling, discharge from the nipple. Paget’s cancer or the so-called eczema-like cancer occupies in mammology only 0.5-5% of cases of neoplasms in the mammary gland. For the first time this disease was diagnosed in 1874 by the scientist Paget, who established the relationship between the development of breast cancer and changes in the nipples; based on observations, it was proved that patients with this pathology develop breast cancer within a year. About half of the patients have a palpable tumor in addition to nipple cancer, more than 90% of cases are characterized by invasive growth, penetration of metastases into the lymph nodes. If there is no tumor, patients are diagnosed with a non-invasive form of the disease.

The content of the article:
Paget’s cancer classification
Causes of Paget’s cancer
Symptoms of Paget’s cancer
Diagnosis of Paget’s cancer
Paget’s Cancer Treatment
Prognosis for Paget’s cancer
Paget ‘s Cancer
Paget’s cancer mainly occurs in patients after the age of 50, although there are cases of detection in 20-year-olds, more often in women. However, Paget’s cancer is more aggressive in men. Since breast cancer is not quite a typical disease for men, the correct diagnosis is made late, which is dangerous for the patient’s life. Paget’s extra-mammary cancer can affect any areas where the apocrine sweat glands are located. In women — on the vulva and perineum, in men — on the penis and scrotum.

Paget’s cancer classification
Based on the variants of the clinical course, Paget’s cancer can occur in various forms. Acute eczema is characterized by the presence of a fine-grained rash, a wet, ulcerated nipple. In the form of chronic eczema, crusts are formed, the removal of which entails the appearance of a wet area. Paget’s cancer, occurring in psoriatic form, forms small pink papules that are covered with peeling dry scales. The ulcerative form is characterized by the formation of ulceration in the form of a crater, and the tumor form is characterized by the presence of a tumor in the mammary gland. In 50% of cases, Paget’s cancer affects only the area of the nipple and areola, in other patients, a palpable tumor node is necessarily detected in the periarticular area. Usually, Paget’s cancer is characterized by a unilateral lesion, but the disease in both nipples is not excluded.

Causes of Paget’s cancer
Until now, medicine has not established the exact cause of Paget’s cancer. There are several theories on this disease. According to the epidermotropic theory, Paget’s cancer cells move along the ducts to the surface of the nipples and begin to grow. This opinion is formed due to the fact that in 95% of cases, together with Paget’s cancer, an intra-current form of breast cancer is detected. Glandular cells from the epithelium of the milky ducts become tumor cells.

Adherents of the transforming theory believe that the neoplasm appears as a result of spontaneous degeneration of healthy nipple and areola cells into pathogenic cells. This hypothesis is suitable for those cases when cancer has not been detected in the breast itself.

Also, genetic predisposition plays an important role in the development of Paget’s cancer, especially in men. Injuries, exposure to carcinogenic substances can be provoking factors of cancer.

Symptoms of Paget’s cancer
Paget’s cancer is insidious in its asymptomatic early stages. Peeling, redness, irritation of the nipple and the periarticular zone are not a reason to contact an oncologist or a mammologist. Moreover, these symptoms spontaneously disappear for a while, especially when using corticosteroid ointments. The disease does not disappear, but flares up with renewed vigor. There is pain and hypersensitivity of the nipple, itching, burning, flattening or retraction of the nipple, serous hemorrhagic discharge. The further the disease progresses, the more pronounced the symptoms appear: stagnant infiltration develops in the area of the nipple and areola, crusts, erosions, ulcers form on the surface. After removing the scales, the exposure of the wet surface is observed.

Eczematous manifestations sometimes capture a part of the skin of the breast, forming a clearly defined, somewhat elevated lesion area. By the method of palpation, 50% of patients experience an increase in lymph nodes, nodular neoplasms in the mammary gland. For the later stages, bloody abundant discharge from the affected area is characteristic.

In men, the symptoms of Paget breast cancer are similar: erythema, peeling, itching, compaction, maceration of the skin, retracted nipple.

Diagnosis of Paget’s cancer
Diagnosing Paget’s cancer is difficult because of the symptoms found in other diseases. It is necessary to differentiate the disease from eczema of the nipples, superficial basal cell carcinoma, melanoma, dermatitis, psoriasis, Bowen’s disease. Herpes, fungal mycosis, tuberculosis, breast syphilis have similar manifestations, so you need to distinguish them from the symptoms of Paget’s cancer. In case of suspicion of Paget’s cancer, it is necessary to conduct comprehensive diagnostic studies, which include examination and consultation of a mammologist-oncologist, mammography, ultrasound, MRI, cytology smear, breast biopsy.

During examination, changes in the nipple are visually observed, nodular tumor formations are felt during palpation. Examination of a smear of discharge from the nipple will help to establish the presence of Paget cells. Under a microscope, you can see large, single, oval or round cells that do not form intercellular bridges, have an enlightened cytoplasm. Around them, epidermal cells undergo compression atrophy. The dermis also changes — it is infiltrated by plasmocytes, hyperemic, new capillaries appear in it, serous exudation, which is a typical clinical picture.

If there is no tumor node, mammography will be effective only by 50%, it should be performed in three projections. An ultrasound examination can be successfully included in the primary examination plan, especially if the mammography data is negative. Therefore, MRI and scintigraphy are the most effective for detecting changes that are not even clinically manifested yet.

Paget’s Cancer Treatment
The main method of treating Paget’s cancer today is radical removal — mastectomy. It is indicated in the case of an invasive form of cancer or with intracurrent carcinoma. The removal of the breast with the pectoral muscles, lymph nodes, and fiber is carried out.

With a non-invasive form of cancer, a simple mastectomy is indicated, the removal of the small pectoral muscle and gland tissue is performed. If the cancer is in the early stages, radical resection is used, which includes the removal of the nipple-areola complex and the tumor with part of the affected gland. For cosmetic purposes, in the future, patients undergo reconstructive mammoplasty.

In addition to surgical treatment, chemotherapy, hormone therapy, and remote gamma therapy are prescribed. The objective of such complex treatment is to prevent metastasis and relapses, especially in young patients. The use of radiation therapy in the early stages and with small lesion sizes allows you to do without much damage to the surrounding tissues. If the progression is of the type of local relapse, then radical surgical intervention remains in reserve at the first stage of treatment. For operable patients, radiation is indicated when more than 4 regional lymph nodes are affected. Radiation therapy is a priority before radical mastectomy and from an emotional position.

In the early localized stages of Paget’s cancer in patients with such unfavorable factors as negative receptors, young age, multicentric growth, high malignancy, it is advisable to prescribe chemotherapy, and if regional lymph nodes are affected, it is necessary.

Prognosis for Paget’s cancer
Unfortunately, it is not always possible to detect Paget breast Cancer in the early stages, so the prognosis almost always depends on timely diagnosis of the disease and treatment. The main significant factors affecting the prognosis for the patient’s life are: the clinical stage, the number of affected lymph nodes, the patient’s age, unfavorable morphological criteria, the degree of malignancy, biological aggressiveness of the disease. If the disease is detected in a timely manner and treatment is carried out successfully, a full recovery is possible.

In the postoperative period, there is a risk of recurrence due to the aggressiveness of the tumor. Life expectancy can be more than 3 years, but its duration is reduced to 1 year if there are metastases and infiltrates.

Only regular self-examination, periodic visits to a mammologist for a thorough examination of the mammary glands can detect Paget’s cancer in the early stages, successfully cope with the insidious disease and significantly affect the prognosis.

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