Paget’s disease of nipple — a form of breast cancer

Paget's disease of nipple is a special type of breast cancer, which is not very common. However, it is the one which can be misdiagnosed easily. It is frequently thought to be a skin inflammation or infection, leading to unfortunate delays in detection and care.
I have seen few patients in Dhaka who had their treatment as nipple eczema for certain length of time and eventually searched for help, as the treatment for eczema did not help at all, instead got worse. After doing punch biopsy of nipple, diagnosis was confirmed as Paget's disease of nipple with underlying invasive breast cancer and had mastectomy(removal of whole breast)!
What is it?
It is an eczema-like change in the skin of the nipple, and 9 out of 10 women who have it have an underlying breast cancer. The underlying breast cancer may be an invasive breast cancer or ductal carcinoma in situ (DCIS). In DCIS, the cancer cells are completely contained within the milk ducts. It is seen in 1-4% of cases of female breast cancer.
How does it present?
* Presents as chronic eczematous change of the nipple with iItching, erythema, scale formation, erosions, nipple discharge including bleeding
* Underlying palpable breast lump (usually indicates an invasive nature).
Investigations
* Punch Biopsy of nipple with immuno-histochemistry and special stains
* Investigation of breast lump if palpable
* Investigations looking for underlying malignancy if no lump palpable, e.g. mammography or MRI (may be more useful) and core biopsy.
Management
The treatment of Paget's disease of the breast will depend on:
* whether or not there is an underlying breast cancer
* whether it is DCIS or an invasive tumour
* how much of the breast is affected.
Surgery
Surgery is the main treatment for Paget's disease. This may involve an operation to remove all of the breast (a mastectomy) or an operation to remove the affected area of breast, including the nipple and areola, and some surrounding normal breast tissue (breast-conserving surgery). Some of the lymph nodes in the underarm may also be removed.
A mastectomy may be recommended if the cancer is affecting a wide area. It may be possible to have breast reconstruction at the same time as a mastectomy or as a second operation some months later.
Some people don't need any further treatment after surgery. Others may need radiotherapy, hormonal therapy or chemotherapy and or immuno-therapy. These treatments may be given separately or together.
Prognosis
Paget's disease of the breast is associated with a poor prognosis, which is worse if the lesion is associated with an underlying palpable mass.
The writer is the Lead Breast Surgeon of United Hospital, Dhaka. E-mail: [email protected]