Paget's cancer is a malignant disease, most often affecting the nipple-areolar complex of the mammary glands.
Since the object of the tumor process with Paget's cancer is always apocrine( large sweat and milk) glands and their ducts, the disease can also develop in the area of axillary hollows, in female and male external genitalia, in the perianal( around the anus) area.
The disease was named after the British pathologist James Paget, who first described the mechanism of its appearance and clinical manifestations.
Concept and statistics of the disease
Paget's disease - eczematoid nipple cancer - accounts for one to five percent of all cases of malignant breast tumors. Thus, it belongs to the category of fairly rare ailments.
The vast majority( 95%) of patients with Paget's disease had breast cancer.
The photo shows the breast cancer Paget's disease
The course of Paget's cancer in men is more aggressive, the rate of leakage and the severity of the consequences.
Specialists explain this by the fact that the mammary glands of men are quite small in size. Tumor cells very quickly affect their tissues, and then, having penetrated into the lymph nodes, rapidly metastasize, spreading throughout the body.
Clinical forms of
Depending on the clinical manifestations, it is customary to distinguish between the following forms of Paget's cancer:
- In the tumor form of , the malignant neoplasm is localized in tissues located directly under the nipple.
- For the psoriatic form of is characterized by the appearance of multiple small pink papules( covered with dry, easily peeling scales) on the outer surface of the nipple.
- With the ulcerative , ulcers of a craterlike appearance appear on the surface of the nipple and the surrounding areola.
- The form, called as an acute eczematoid , is characterized by the formation of a small rash, followed by wetting ulcers.
- For the of the chronic eczematoid , the formation of crusts on the surface of the nipple and areola is characteristic. The removal of crusts leads to the detection of wet eczematous areas.
Half of the patients have lesions only in the nipple and its areola. In 40% of cases, in addition to the above symptomatology, a tumor is detected in the form of a knot that can be palpated.
The remaining 10% of the episodes of Paget's cancer are detected quite accidentally( as a result of a laboratory study of the discharge taken from the nipple).It is after their appearance that the patient turns to the doctor.
Reasons for
There are no reliable reasons for the occurrence of Paget's cancer to this day. In modern mammology, there are two points of view, trying to give an answer to this question.
- Followers of the epidermotropic theory believe that in the presence of breast cancer( localized in its deepest layers), cancer cells called Paget cells from the tumor tissues enter the milky ducts and migrate to them to reach the nipple. Implanting in his tissues, cells begin to expand. The basis for this theory is observations confirming the fact that 95% of patients, along with Paget's cancer, have other types of breast cancer( most often intraprostate carcinoma).Paget's cancer cells, localized in the tissues of the nipple and formed by the epithelium of the milk ducts, are glandular in nature.
- Supporters of transforming the theory of believe that the cause of the development of Paget's cancer is the spontaneous degeneration of the epithelial tissues of areola and nipple into cancerous ones. The basis for this hypothesis are cases of Paget's cancer, not accompanied by malignant tumors of other localization.
Among the risk factors contributing to the occurrence of Paget's cancer, the researchers include:
- Presence of family predisposition to this disease( especially in the male line).
- Exposure to carcinogens contained in the environment.
- All possible injuries of the nipple-areola zone.
Paget Cancer Symptoms
- Early symptomatology of Paget's disease, which is not alarming or specific, does not cause the patient to seek medical help. The onset of the disease is marked by a slight reddening, peeling and slight irritation of the skin of the parotid zone and the nipple itself. The tendency to the sudden disappearance of the above manifestations, calming the patient, only aggravates the situation, since the disease, which does not manifest itself, continues to develop in its organism. Sometimes eczematous changes can be eliminated with ointments containing corticosteroids.
- Progression of the disease makes you aware of the occurrence of itching, tingling, burning sensation, increased sensitivity and soreness of the nipple. From it, serous hemorrhagic discharge begins to appear, leaving traces on the linen. The nipple configuration also changes: it can flatten or retract.
- With further progression of Paget's cancer in the nipple tissues and its areola, hyperemia and stagnant infiltration are observed. On the surface of the areola, there are all kinds of erosions, ulcers, dry crusts. When trying to remove crusts, areas of shedding flesh are exposed. Paget's cancer is most often characterized by a one-sided lesion, but in medical practice, there are cases of bilateral nature, in which the tumor affects the nipples of both mammary glands.
- Eczematous manifestations can spread to the skin of the mammary gland .Clearly delineated problem area, as a rule, rises somewhat above healthy skin. In 50% of patients palpation reveals the presence of nodal neoplasms in the tissues of the mammary glands and an increase in a number of regional lymph nodes.
- The late stage of Paget's cancer is characterized by the presence of copious bleeding , caused by destruction( destruction) of the nipple-areolar complex.
Signs of Paget's cancer in men have similar manifestations.
Methods of diagnosis
Timeliness of the correct diagnosis is the key to a successful fight against a malignant disease.
To detect Paget's cancer, a whole complex of laboratory and hardware studies is needed:
- In the process of visual inspection, a specialist mammologist can detect a number of changes in the nipple and its areola, which are characteristic for this pathology( the presence of ulcers, dry crusts, wetting specimen).
- If there is a discharge from the nipple, the patient is given a smear and subjected to a cytological examination. It will definitely detect cancer cells.
- A mammogram for patients with suspected Paget's cancer is performed in three projections at once: this allows to exclude the presence of a malignant tumor of a different type. The method of mammography is most informative in the presence of tumor formations in the mammary glands. If they are absent, the reliability of the results of the obtained mammogram is not more than 50%.
- Ultrasound examination of mammary glands in the initial diagnosis of Paget's cancer is a mandatory procedure, as its data often reveal pathologies that are not reflected by a mammogram.
- Magnetic resonance imaging allows not only to detect the disease at the earliest stages( even in the absence of visible clinical disorders), but also to differentiate detected tumors.
- The method of scintigraphy( based on the introduction of radioactive isotopes into the patient's organism and subsequent evaluation of the results of their distribution in tissues) allows detecting tumors of the smallest size, which is very important for early diagnosis of cancer.
- For the detection of cancer cells, a biopsy is used - a laboratory study of tissues taken from the nipple and the areola area.
- The leading method of treating Paget's cancer is surgical intervention. When combined with intracapsular carcinoma or in the presence of an invasive form, a radical mastectomy is performed, consisting in the removal of the mammary gland together with the tissues of the cellulose, lymph nodes and pectoral muscles.
- If Paget's cancer is caught at an early stage, a radical breast resection consisting of removing a part of the breast containing the tumor together with the nipple-areolar complex is performed. After a while, the patients go through a reconstructive mammoplasty operation.
Treatment of breast
Treatment of Paget's cancer is characterized by a systematic approach aimed at preventing relapse of the disease and preventing the occurrence of metastases, therefore surgical treatment is necessarily accompanied by chemotherapy, radiotherapy and hormone therapy.
- Radiation therapy is used in all stages of Paget's cancer. In the presence of absolute contraindications to surgery( especially in the old age), it can be used as an alternative, replacing the operation.
- Indications for the mandatory appointment of chemotherapy is the defeat of regional lymph nodes.
- Hormonotherapy( treatment with nolvadex, tamoxifen, zitazonium) is only used when detecting receptors that are sensitive to steroid hormones. The appointment of such drugs to young patients is carried out only after blocking the work of the ovaries. Women, whose age falls at the menopause, taking drugs prescribed without affecting the ovaries.
Prognosis and prevention of
Pregnancy prognosis for Paget's cancer depends on a number of factors affecting it:
- clinical stage of the disease;
- of the patient's age;
- the number of affected lymph nodes;
- the presence of aggravating morphological features;
- is a multiplicity of cancers or a combination of a disease with infiltrative lobular cancer.
Prevention of Paget's cancer is reduced to regular visits to a specialist mammologist and periodic diagnostic examination of the mammary glands. Timely detection of malignant neoplasm and treatment at an early stage significantly improves the patient's prognosis and chances of survival.
Video shows a radical mastectomy with preservation of pectoral muscles in left breast cancer Paget: