Paget’s carcinoma is a very rare type of cancer that usually manifests in the nipple (breast) area, but can also occur in other areas of the body. The disease is usually associated with other deeper epithelial carcinomas. Complete cure can often be achieved with early treatment.
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 focus of inflammation in the area of the nipples. In very rare cases, it also occurs outside the breast in the area of the genitals, anus or armpits. In about two thirds of cases it develops from a carcinoma in situ, but especially from a ductal carcinoma in situ. In one third of patients, it accompanies ductal carcinoma of the breast. A carcinoma in situ is a tumor that is still in place and shows no signs of extension. This can therefore also be referred to as precancerous. 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, in addition to 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 by the synonyms Paget’s cancer, Paget’s disease, Paget’s disease of the nipple, dermatitis papillaris maligna, or cancerous eczema of the breast. Not all synonyms do justice to the fact that Paget’s carcinoma can occasionally be located in the extramammary region. 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. There is a theory that it develops from cancer cells that originally reside in the milk ducts of the breast and migrate from there to the nipples. This theory was derived from the observation that Paget’s carcinoma is always located on the same side of the breast carcinoma. Other explanations assume that the cancer cells originate directly at the nipples or areola. However, it is possible that both theories are correct. This is because Paget’s carcinoma is usually, but not always, associated with breast carcinoma or its precursor. It also occasionally occurs in isolation or even in the extracellular space.
Symptoms, complaints, and signs
Paget’s carcinoma is characterized by an eczema-like rash on the nipples or genitals. There is an intermittent serous discharge and weeping crust formation there. Pain is not felt. The nipple and its surroundings are reddened. In addition, skin thickening, edema and inflammatory changes occur in this area. An unmistakable sign of the presence of Paget’s carcinoma is the retraction of the nipple. However, this finding again suggests the presence of ductal breast carcinoma or ductal carcinoma in situ. Even when Paget’s carcinoma occurs in the extramammary region, it may be caused by migrating cancer cells from an underlying tumor. The extramammary form of Paget’s carcinoma is also characterized by eczema-like skin lesions.
Diagnosis and disease progression
The diagnosis of Paget’s carcinoma first requires an extensive medical history. Among other things, this involves asking about the time of onset of symptoms and any changes in them. Furthermore, it is important for the physician to know what medications the patient is taking and whether similar disease symptoms have already occurred within the family or relatives. The physician 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. Retraction of the nipple indicates breast carcinoma. Examinations include mammography, MRI, and CT scan. In addition, the breast and axilla should be palpated and a biopsy should be performed. Paget’s carcinoma, clearly diagnosed, provides an indication of underlying ductal breast carcinoma or ductal carcinoma in situ.The success of treatment depends on a clear diagnosis of the original carcinoma.
Complications
Since this disease is a 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 in this case, so that the patient’s life expectancy is also not reduced by the disease. As a rule, patients suffer from a skin rash due to Paget’s carcinoma. This can significantly reduce the patient’s appearance, leading to decreased self-esteem or inferiority complexes. A retraction of the nipple or a discharge on the patient’s genitals may also indicate this disease. In many cases, however, those affected also suffer from another tumor. As a result, the patient suffers from permanent fatigue and lassitude. The patient’s quality of life is significantly reduced by Paget’s carcinoma. The tumor can be removed with the help of a surgical procedure. Complications do not occur. However, patients are still dependent on chemotherapy to completely defeat the cancer. Usually, the life expectancy of the affected person is not reduced if the therapy is successful.
When should you see a doctor?
Changes in skin appearance or the development of eczema should be presented to a physician. Characteristic of Paget’s carcinoma are abnormalities of 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 clarified by a physician. Since the disease can lead to premature death if left untreated, a visit to the doctor is advisable at the first sign of any irregularity. Treatment as soon as possible is necessary to avoid complications or spread. A doctor is needed if there is an internal disturbance, weeping areas on the body and redness. Open areas must be treated sterilely. There is a risk of sepsis, which is an acute health-threatening condition. If the affected person is unable to provide adequate wound care under his or her own power, or if irregularities occur in the healing process of an open wound, a physician must be consulted. Decreases in libido, well-being and general quality of life should be discussed with a physician. If behavioral abnormalities or mood swings as well as dullness occur, a check-up visit to a physician is advisable. Malaise, difficulty concentrating, and a feeling of illness are other signs of impaired health that should be discussed with a physician.
Treatment and therapy
If treatment begins early, a complete cure can be achieved. In the early stage of the disease, cancer growth is still limited, so surgical removal of the carcinoma is sufficient. In this case, the affected nipple and areola are removed. Chemotherapy or radiotherapy is then often not yet necessary. This is especially true for 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. Furthermore, radiation of the lymph drainage area and chemotherapy must then follow. According to experience, the chances of recovery are good in more than 80 percent of cases.
Outlook and prognosis
Because Paget’s carcinoma is mostly caused by a deeper-lying carcinoma, the prognosis depends largely on the stage and type of this associated tumor. A significant basis for good prospects of cure is early diagnosis of symptoms, primarily eczema-like changes in the area of the female nipples. If the tumor is diagnosed at an early stage, complete recovery can be achieved by surgical removal of the affected tissue. Breast conservation measures and breast reconstruction with autologous tissue or implants are safe from a health point of view. Diagnosis at a late stage significantly reduces the prospects of recovery, as it must be assumed that daughter tumors (metastases) have formed.The prognosis depends largely on the number of affected lymph nodes (lymph node status). If malignant cancer tumors have to be treated with chemotherapy, the chances of recovery are difficult to predict. The drugs used are cytostatics, which inhibit not only cancer cells but also other body cells that divide frequently. The associated side effects, such as hair loss and weakening of the immune system, can have a serious impact on quality of life. The treatment of Paget’s carcinoma leads to a considerable psychological burden for those affected and a diminished sense of self-worth. Measures to strengthen the psyche through professional care and intensive care by relatives are essential for mental recovery.
Prevention
There is no real prevention of Paget’s carcinoma. Only the risks can be somewhat reduced. This includes a healthy lifestyle, reducing excess weight and abstaining from 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 persistent and weeping eczema with crusting and scaling around the nipples or genitals.
Follow-up
As a rule, the measures or options for aftercare in Paget’s carcinoma are significantly limited or not available to the affected person at all. In this regard, early diagnosis of this disease is particularly necessary to prevent complications or further spread of the tumor as it progresses. Self-healing is not possible with this disease. Most of the affected persons are dependent on a surgical intervention, whereby usually also the measures of a radiation therapy or a chemotherapy are necessary. Regular check-ups by a physician are also very important after removal in order to detect and treat further tumors at an early stage. Most patients with Paget’s kidney Paget’s carcinoma are also dependent on the permanent support and care of their own family. This can alleviate or even completely prevent the development of depression and other psychological upsets. Whether Page’s kidney Paget’s carcinoma leads to a reduced life expectancy of the affected person cannot be universally predicted. The earlier the disease is detected, the better is usually the further course.
What you can do yourself
The disease leads to a strong psychological and emotional sense of pressure in many patients. It is therefore particularly important to promote a zest for life. Despite all adversity 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 of the disease, the patient should also deal with topics that can distract him from the 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 sufferers 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 arises, as a comprehensive understanding is provided by both sides. To stabilize one’s own health, it is important that a strengthening of the immune system takes place. A balanced diet rich in vitamins can help minimize side effects. In addition, stays of fresh air are important for the organism. The consumption of harmful substances such as nicotine or alcohol should be completely avoided. Sufficient sleep and good sleep hygiene also strengthen the body.
