Paget's disease of the nipple is a rare but dangerous form of breast cancer, accounting for 1 to 3% of all breast tumours. Insidious, it often disguises itself as a simple skin irritation, dangerously delaying diagnosis, even though it is associated with an underlying breast tumour in 80% of cases. Warning signs, diagnostic tests, treatment options and prognosis: here is what you need to know about this rare condition.
This insidious form of breast cancer in alarming figures
Paget's disease of the nipple is a rare but dangerous form of breast cancer, accounting for 1 to 3% of all breast tumours. At the Regional Oncology Centre of Tangier in Morocco, this condition reveals its insidious nature: it often disguises itself as a simple skin irritation, dangerously delaying diagnosis.
More worrying still, this disease is associated with an underlying breast tumour in 80% of cases. In Tunisia, where the Salah Azaiez Institute treats many cases of breast cancer, doctors stress the importance of early diagnosis of this particular form.
Paget's disease of the nipple: when the skin betrays the cancer
This condition results from the invasion of the nipple epidermis by a ductal carcinoma in situ. Contrary to popular belief, it is not the skin that becomes cancerous first. The malignant cells migrate from the deep milk ducts towards the surface of the nipple skin.
The deceptive appearance of this disease is what makes it dangerous. It begins as a simple redness resembling eczema. This lesion may take on a crusty, scaly appearance and cause burning sensations, itching or pain. Unfortunately, this ordinary presentation often delays diagnosis.
Warning signs never to ignore
Recognising the symptoms of Paget's disease of the nipple can save lives. Several signs should raise the alarm immediately:
Skin changes of the nipple
- Persistent crusting or peeling of the skin
- Localised redness that does not heal
- An eczema-like appearance that resists conventional treatments
Worrying physical symptoms
- Pain, itching or burning sensations
- Abnormal discharge or bleeding from the nipple
- A change in the appearance or shape of the nipple (retraction inwards)
Signs that the disease is spreading
- A palpable lump in the breast
- A swollen lymph node under the armpit
- Spread of the lesions towards the areola or surrounding skin
A crucial detail: these symptoms generally affect a single breast and persist for more than three weeks. In Algeria, where only three cancer-care centres exist for the entire country, this early recognition becomes vital.
Diagnosing Paget's disease of the nipple: essential examinations
Faced with suggestive symptoms, several examinations will confirm the diagnosis. First, a specialist consultation is needed promptly. The oncologist will carry out a full clinical examination including breast palpation and examination of the lymph node areas.
Essential imaging examinations
The mammogram and breast ultrasound are the first diagnostic step. Be aware, however: these examinations can sometimes be normal without ruling out the diagnosis.
A breast MRI proves particularly valuable for detecting an underlying cancer and carrying out a full work-up before treatment.
Biopsy: the examination that confirms
A skin biopsy remains the essential examination for making a definitive diagnosis of Paget's disease of the nipple. Histological analysis reveals the presence of characteristic cells: large pale cells with large nuclei infiltrating the epidermis.
Immunohistochemistry completes this analysis by showing cells positive for cytokeratin CK7 and negative for CK20, often with overexpression of HER2.
Groundbreaking treatments for Paget's disease of the nipple
Management depends essentially on whether or not there is an associated underlying breast cancer. Fortunately, the treatment options are becoming more varied and are improving.
Surgery: the cornerstone of treatment
Surgical treatment remains the standard for Paget's disease of the nipple. Two main approaches exist:
Conservative surgery A nipple-areola excision consists of removing only the nipple-areola complex and the underlying breast tissue. This option preserves the breast when the disease remains localised.
Extended surgery In the case of an extensive cancer or a large underlying tumour, a mastectomy becomes necessary to eliminate the disease completely.
In all cases, lymph node dissection often accompanies the surgical procedure.
Innovative complementary treatments
Several therapies complement surgery depending on the characteristics of each patient:
Adjuvant radiotherapy It systematically follows conservative surgery to eliminate any residual cancer cells and reduce the risk of recurrence.
Targeted chemotherapy Tailored to the molecular profile of the tumour, particularly effective in HER2-positive forms.
Hormone therapy Indicated when hormone receptors are positive, it blocks the hormonal stimulation of the cancer cells.
Immunotherapy This groundbreaking approach mobilises the body's natural defences against the cancer cells.
| Treatment approach | Indication / description |
|---|---|
| Conservative surgery (nipple-areola excision) | Removes the nipple-areola complex and underlying tissue; preserves the breast if the disease remains localised |
| Extended surgery (mastectomy) | Necessary in the case of an extensive cancer or a large underlying tumour |
| Adjuvant radiotherapy | Systematically follows conservative surgery to reduce the risk of recurrence |
| Targeted chemotherapy | Tailored to the molecular profile of the tumour, particularly effective in HER2-positive forms |
| Hormone therapy | Indicated when hormone receptors are positive |
| Immunotherapy | Mobilises the body's natural defences against the cancer cells |
Prognosis and hope in the face of Paget's disease of the nipple
The prognosis depends largely on whether there is an associated underlying breast cancer and on the stage at diagnosis. Diagnosed early, with no associated tumour, Paget's disease of the nipple is treated effectively with excellent results.
Nevertheless, the presence of an associated invasive carcinoma significantly influences the prognosis, requiring more complex management.
Specific challenges in the Maghreb and Africa
Access to cancer care remains problematic in the region. Morocco has only six state oncology centres, Algeria three cancer-care centres, and Tunisia a single specialised anti-cancer centre.
This situation explains why many patients have to travel to the major cities or sometimes abroad to receive optimal care.
Leading regional organisations
Tunisia
- Salah Azaiez Institute of Tunis (reference centre)
- Tunisian Society of Medical Oncology (STOM)
- Tunisian Society of Oncology and Radiotherapy (STOR)
Algeria
- Pierre and Marie Curie Centre (Algiers, Oran, Blida)
- Algerian Society of Medical Oncology
- Cancer surveillance laboratory (Oran)
Morocco
- Mohammed VI Centre for Cancer Treatment (Casablanca)
- International Oncology Centre of Casablanca
- Pasteur Institute of Morocco (cancer research)
Prevention and screening: your best weapon
Although there is no specific way to prevent Paget's disease of the nipple, regular breast monitoring remains crucial. Monthly self-examination, annual gynaecological check-ups and screening mammograms are your best assets.
Above all, never hesitate to seek advice at any persistent change in the appearance of the nipple. This vigilance can make the difference between an early diagnosis and complex management.
Paget's disease of the nipple remains a rare but serious condition. Its resemblance to benign skin conditions is its main danger. Fortunately, when diagnosed in time, it is treated effectively thanks to current therapeutic advances. The key to success lies in the early recognition of symptoms and prompt access to specialised care.
