Paget’s carcinoma is a very rare type of cancer that usually manifests itself in the nipple area, but can also occur in other parts of the body. The disease is usually associated with other underlying epithelial carcinomas. Early treatment can often lead to complete healing.
What is Paget’s carcinoma?
Paget ‘s carcinoma occurs in about two to three percent of women affected by breast cancer. It is characterized by an itchy and scaly inflammatory focus in the nipple area. In very rare cases, it also occurs outside the breast in the area of the genitals, anus or armpits. See gradinmath for What is PA.
In about two-thirds of cases, it develops from carcinoma in situ, but particularly from ductal carcinoma in situ. In one third of the patients, it accompanies a ductal breast carcinoma. A carcinoma in situ is a tumor that is still in place and shows no signs of spreading. This can therefore also be referred to as a precancerous condition.
The term “ductal” indicates that the milk ducts of the breast are affected. However, men can also develop Paget’s carcinoma. In men, too, one or both nipples are usually affected, along with other sites of manifestation. Paget’s carcinoma was first described in 1874 by the English surgeon and pathologist James Paget.
The disease is also known under the synonyms Paget’s cancer, Paget’s disease, Paget’s disease of the nipple, dermatitis papillaris maligna or cancer eczema of the breast. Not all synonyms do justice to the fact that Paget’s carcinoma can occasionally be found in the extramammary area. However, Paget’s carcinoma should not be confused with the so-called Paget’s syndrome, which describes a disease of the skeletal system and was also described by James Paget.
Causes
The exact cause of Paget’s carcinoma is not yet fully understood. It is theorized to develop from cancer cells that originally reside in the milk ducts of the breast and from there migrate to the nipples. This theory was derived from the observation that Paget’s carcinoma is always on the same side as breast carcinoma.
Other explanations assume that the cancer cells originate directly in the nipples or areola. However, both theories may be correct. Although Paget’s carcinoma is usually, but not always, associated with breast cancer or its precursors. It also occasionally occurs in isolation or even in the extracellular domain.
Symptoms, Ailments & Signs
Paget’s carcinoma is characterized by an eczema -like rash on the nipples or genitals. At times there is a serous discharge and weeping crust formation. Pain is not felt. The nipple and its surroundings are red.
In addition, skin thickening, edema and inflammatory changes in this area occur. An unmistakable sign of the presence of Paget’s carcinoma is the retraction of the nipple. However, this finding again points to the presence of ductal breast carcinoma or ductal carcinoma in situ.
Even when Paget’s carcinoma occurs in the extramammary area, it can be triggered by migrating cancer cells from an underlying tumor. The extramamillary form of Paget’s carcinoma is also characterized by eczema-like skin changes.
Diagnosis & course of disease
The diagnosis of Paget’s carcinoma first requires a detailed medical history. Among other things, questions are asked about when the symptoms started and how they changed. Furthermore, it is important for the doctor to know what medication the patient is taking and whether similar symptoms have already occurred within the family or relatives.
The doctor is also interested in possible hereditary diseases, allergies or possible stress conditions. This information makes it possible to exclude other diseases such as normal skin eczema, allergies or parasitic infestation of the skin. Nipple retraction indicates breast cancer.
Tests include mammography, MRI, and CT. In addition, the breast and armpits should be palpated and a biopsy performed. A clearly diagnosed Paget’s carcinoma provides an indication of an underlying ductal breast carcinoma or a ductal carcinoma in situ. The success of the treatment depends on the clear diagnosis of the original carcinoma.
Complications
Since this disease is a form of cancer, the further course depends very much on the time of diagnosis and the spread of the tumor. With early diagnosis and treatment, further complications can be avoided relatively well, so that the patient’s life expectancy is not reduced by the disease. As a rule, patients suffer from a rash due to Paget’s carcinoma.
This can significantly diminish the patient’s appearance, leading to reduced self-esteem or an inferiority complex. A retraction of the nipple or a discharge on the patient’s genitals can also indicate this disease. In many cases, however, those affected also suffer from another tumor. This leads to permanent tiredness and exhaustion of the patient.
The patient’s quality of life is significantly reduced by Paget’s carcinoma. The tumor can be removed with a surgical procedure. There are no complications. However, those affected are still dependent on chemotherapy to completely defeat the cancer. As a rule, the life expectancy of those affected is not reduced if the therapy is successful.
When should you go to the doctor?
Changes in the complexion or the development of eczema should be presented to a doctor. Characteristic of Paget’s carcinoma are abnormalities in the skin on the nipples or genitals of the affected person. Thickening of the upper layers of the skin, inflammation and discoloration of some areas of the skin should be examined. A rash, itching or the formation of crusts should be checked by a doctor. Since the disease can lead to premature death if left untreated, it is advisable to see a doctor at the first sign of an irregularity.
Prompt treatment is necessary to avoid complications or spread. In the case of inner restlessness, weeping areas on the body and redness, a doctor is needed. Open areas must be treated sterile. There is a risk of sepsis, which is an acute health-threatening condition. If the person concerned is unable to provide adequate wound care on their own or if there are irregularities in the healing process of an open wound, a doctor must be consulted.
Decreases in libido, well-being and general quality of life should be discussed with a doctor. If there are any abnormalities in behavior or if there are mood swings or fatigue, it is advisable to see a doctor for a check-up. Feeling unwell, having trouble concentrating, and feeling ill are other signs of a health condition that should be discussed with a doctor.
Treatment & Therapy
If treatment is started early, a complete cure can be achieved. In the early stages of the disease, cancer growth is still limited, so surgical removal of the carcinoma is sufficient. The affected nipple and areola are removed. Chemotherapy or radiation therapy is then often not necessary.
This is especially true in cases with ductal carcinoma in situ. In these cases, breast-conserving surgery is also possible. However, if the carcinoma has already spread and spread, a mastectomy with breast reconstruction measures is usually necessary. In addition, radiation of the lymphatic drainage area and chemotherapy must then be added. According to experience, the chances of recovery are good in over 80 percent of cases.
Outlook & Forecast
Since Paget’s carcinoma is usually caused by an underlying carcinoma, the prognosis depends significantly on the stage and type of this associated tumor. An important basis for good prospects of healing is an early diagnosis of the symptoms, primarily of eczema-like changes in the area of the female nipples. If the tumor is diagnosed at an early stage, full recovery can be achieved by surgically removing the affected tissue. Breast-conserving measures and breast reconstructions with autologous tissue or implants are harmless to health.
Diagnosis at a late stage reduces the chances of a cure considerably, as it must be assumed that secondary tumors (metastases) have formed. The prognosis depends largely on the number of affected lymph nodes (lymph node status). If malignant cancerous growths have to be treated with chemotherapy, the chances of recovery are difficult to predict. The drugs used are cytostatics that not only inhibit cancer cells, but also other body cells that divide frequently. The associated side effects, such as hair loss and the weakening of the immune system, can have a serious impact on quality of life.
The treatment of Paget’s carcinoma leads to considerable psychological stress for those affected and reduced self-esteem. Measures to strengthen the psyche through professional care and intensive care by relatives are essential for mental recovery.
Prevention
Real prevention of Paget’s carcinoma is not possible. Only the risks can be reduced somewhat. This includes a healthy lifestyle, reducing excess weight and avoiding alcohol and smoking. If there is a family history of breast cancer, the breast should be examined regularly by a gynecologist.
If skin changes occur in the breast or genital area, a doctor should be consulted immediately. These skin changes include stubborn and weeping eczema with crusting and flaking in the nipple or genital area.
Aftercare
As a rule, the measures or the options for aftercare in Paget’s carcinoma are significantly limited or are not available to the person concerned. Above all, an early diagnosis of this disease is necessary so that there are no complications or further spread of the tumor in the further course. Self-healing cannot occur with this disease.
Most of those affected are dependent on an operative intervention, whereby the measures of radiotherapy or chemotherapy are usually also necessary. Regular check-ups by a doctor are also very important after the removal in order to identify and treat further tumors at an early stage. Most patients with Paget’s kidney also depend on the ongoing support and care of their own family.
This can alleviate or even completely prevent the development of depression and other mental disorders. It cannot be universally predicted whether Paget’s kidney disease will lead to a reduced life expectancy for those affected. The earlier the disease is detected, the better the further course is, as a rule.
You can do that yourself
The disease leads to a strong psychological and emotional feeling of pressure in many patients. It is therefore particularly important to promote joie de vivre. Despite all adversities and unpleasant circumstances, leisure activities are necessary that lead to a strengthening of well-being.
In addition to comprehensive information about the symptoms, the course of the disease and the treatment options for the disease, the patient should also deal with topics that can distract him from current developments. The use of mental techniques can help to strengthen the psyche. This is an essential part of dealing with the disease.
The exchange and contact with other patients can be perceived as pleasant. Open questions can be addressed and clarified. The conversations with other patients are described by many as very intimate and helpful. Mutual support develops as a comprehensive understanding is brought up by both sides.
In order to stabilize one’s own health, it is important that the immune system is strengthened. A balanced diet rich in vitamins can help minimize side effects. In addition, staying in fresh air is important for the organism. The consumption of harmful substances such as nicotine or alcohol must be completely avoided. Adequate sleep and good sleep hygiene also strengthen the body.
