Paget's disease of the breast
At present time, there is an increasing awareness in the society about breast cancer, which is the second most common cancer among Bangladeshi women. Paget's disease, an extremely rare variety of breast cancer, can affect both female and male population. The danger is in the fact that the disease presents with skin surface changes in and around the nipple and hence may be ignored by the patient for a long time.
The symptoms of Paget's disease may include itching, tingling or redness in the nipple and/or areola; flaking, crusty or thickened skin on or around the nipple; a flattened nipple; discharge from the nipple that may be yellowish or bloody — these are often mistaken for those of some benign skin conditions like dermatitis or eczema. Because the early symptoms of Paget's disease mimic a benign skin condition and because the disease is rare, it has every risk of being misdiagnosed at first, ultimately delaying the diagnosis.
Only a biopsy can confirm the diagnosis; various types of biopsy, like needle biopsy, wedge biopsy or punch biopsy may be used. Since most people who have Paget's disease of the breast also have one or more tumours inside the same breast, in addition to a nipple biopsy, the surgeon performs a clinical breast exam to check for lumps or other breast changes.
As many as 50% of people who have Paget's disease of the breast have a breast lump that can be felt in a clinical breast exam. The surgeon may ask for additional diagnostic tests.
For many years, mastectomy, with or without the removal of lymph nodes under the arm on the same side of chest was regarded as the standard surgery for Paget's disease of the breast.
However, that breast-conserving surgery that includes removal of the nipple and areola, followed by whole breast radiation therapy, is a safe option for people with Paget's disease who do not have a palpable lump in their breast and whose mammograms do not reveal a tumour.
The prognosis of the disease depends on various factors — mainly delay in detection, age of the patient, spread of the disease, whether invasive cancer is present or not and presence of other tumours in the same breast. The presence of invasive cancer in the affected breast and the spread of cancer to nearby lymph nodes are associated with reduced survival.
An 80-year old Bangladeshi lady was admitted in United Hospital with the complaints of ulceration and pain in the left breast for last one year. On examination, it was seen that the left nipple and areola has been completely replaced by a large ulcer which has even spread beyond the areola. A clinical diagnosis of breast cancer (Paget's disease) was made which was subsequently confirmed by biopsy. After proper evaluation and consultation, mastectomy with axillary clearance under general anaesthesia was performed. This was well tolerated by the patient and she had an uneventful recovery.
My experience shows that Paget's disease of the breast, although rare, is not uncommon in Bangladesh.
The writer is a Consultant of General, Laparoscopic & OncoSurgery working at United Hospital Limited, Dhaka.
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