Paget’s disease of the nipple is linked to breast cancer that particularly affects women over the age of 50. It is a rare disease. Symptoms, evolution, treatment, healing, survival: discovery.
There Paget’s disease of the nipple East a rare form of breast cancer which mainly affects women over 50, after menopause. The young woman is very rarely affected. Health authorities estimate that only 1 to 3% of breast tumors are Paget’s diseases of the nipple. What are the symptoms of this disease? The nipple itches? How to cure it? What survival?
What is Paget’s disease of the nipple?
“The definition of Paget’s disease is histological. It corresponds to the presence of abnormal cells (Paget cells) in the skin of the nipple” defines Dr. Enora Laas, surgeon in senological, gynecological and reconstructive surgery at the Institut Curie. This rare variety of ductal carcinoma in situ is manifested by an eczematous nipple that begins to bleed and scratch. “In more than 80% of cases, Paget’s disease is associated with underlying breast cancer.“, informs Dr. Enora Laas. Paget’s disease of the nipple should not be confused with another pathology simply called “Paget’s disease of bone” and which corresponds to bone damage with increase in size and deformation of certain bones.
Photo of Paget’s disease of the nipple
Paget’s disease can have other localizations, in the groin or around the vulva (vulvar Paget’s disease). It is a rare adenocarcinoma that can arise from the apocrine glands in the skin or from cancer of the bladder, anus or rectum.
What are the symptoms of Paget’s disease of the nipple?
Nipple cancer manifests usually on only one nipple. “Paget’s disease of the nipple is manifested by an eczema-like lesion on the nipple that is itchy. The nipple may bleed” describes Dr. Laas. The extension of the lesion is from the center of the nipple towards the areola. The symptoms of Paget’s disease of the nipple are, to summarize:
- Sores on the nipple (which looks like eczemathis is called an eczematous lesion of the nipple)
- a nipple itchy
- a nipple who bleeds
What is the prognosis for survival?
Breast Paget’s disease, alone or associated with adenocarcinoma in situ, has a 15-year survival rate close to 90%while it is 60% when it is associated with invasive adenocarcinoma, indicates an article by the International Journal of Medical Reviews and Case Reports (2020).
What causes Paget’s disease of the nipple?
In more than 80% of cases, Paget’s disease of the nipple is associated with cancer of the mammary gland. Paget’s disease of the nipple mainly affects women after menopause. The average age at diagnosis is 51 to 70 years old according to studies.
Paget’s disease of the nipple in young women?
Paget’s disease of the nipple occurs in postmenopausal women in 2/3 of cases. Very rarely in young women (under 40).
“Diagnosis of Paget’s disease of the nipple is made with clinical examination and biopsy to confirm the diagnosis. Sampling for analysis is done under local anesthesia during a consultation“Informs Dr. Enora Laas. When the diagnosis is confirmed, we look for the underlying cause, breast cancer in the majority of cases. “A mammogram, breast ultrasound, or even an MRI will be performed to find breast cancer“explains the surgeon. In the event of the presence of an underlying cancer of the mammary gland, an extension report that is to say the realization of complementary examinations to see the presence of cancerous cells having invaded other organs, is sometimes necessary for the decision of the best therapy to adopt.
What is the treatment for Paget’s disease of the nipple?
The treatment of nipple cancer depends on whether or not it is associated with an underlying cancer and on the advancement of the tumor. “The treatment is surgical and consists of remove the nipple and the gland under the nipple and removing any associated cancer which is often cancer in situexplains Dr. Enora Laas. We realize a sentinel lymph node (removal of one or two lymph nodes for analysis). A post-operative radiotherapy is performed to sterilize the breast after surgery. If the cancer is aggressive, there may be chemotherapy“If the patient prefers a total mastectomy (removal of the breast), radiation therapy is not necessary.”The nipple can be reconstructed with a graft or a tattoo“says Dr. Laas.
What prevention to avoid Paget’s disease of the nipple?
“Prevention of Paget’s disease of the nipple consists of an annual examination by a gynecologist and by a mammogram from the age of 50“says Dr. Enora Laas. Organized screening for breast cancer concerns all women between 50 and 75 years old. It is done by a mammogram of both breasts, every two years.”It is also appropriate to consult in the presence of a lesion or pruritus (itching) of a nipple that persists” recommends the surgeon.
Thanks to Dr. Enora Laas, surgeon in senological, gynecological and reconstructive surgery at the Institut Curie.
Source : Salaheddine Achkif et al./ International Journal of Medical Reviews and Case Reports (2020) 4(10):132-134