Breast cancer

Updated 13 November 2017

Spot rare breast cancers

An e-mail warning women of a 'new kind of breast cancer' has been doing the rounds lately. We put together the facts on two of the rarest forms of breast cancer.

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An e-mail warning women of a 'new kind of breast cancer' has been doing the rounds lately. It relates the tragic tale of a woman who has apparently since died as a result of the cancer, known as Paget's disease of the nipple.

According to the e-mail, the woman first noticed a change in her nipple when a rash appeared on it and after undergoing treatment successfully, the cancer resurfaced in her liver a year later, which subsequently killed her.

In the e-mail the woman pleads with other women to 'be alert to anything that's not normal' and to 'be persistent in getting help as soon as possible'.

What is Paget's disease of the nipple?

Paget’s disease of the nipple (not to be confused with Paget's disease which is a bone disorder) is a rare type of breast cancer, which occurs mainly in older women, and appears as a rash on the nipple.

The changes in the nipple usually indicate that there is an underlying cancer in the breast ducts. This is often just under the nipple and has extended into the nipple. This sign of breast cancer may indicate a non-invasive area of ductal carcinoma in situ (DCIS).

In other cases, Paget’s disease of the nipple may indicate an invasive cancer in another part of the breast. Some women who come to the doctor with a nipple rash will have a lump in the breast which can be found by physical examination or mammogram.

Rare forms of breast cancer
In the interests of clarity and accuracy, Professor Justus Apffelstaedt (Associate Professor, University of Stellenbosch and Head: Breast Clinic, Tygerberg Hospital), put together the following table to compare two of the rarest forms of breast cancer; Paget's disease of the nipple and inflammatory breast cancer.

Inflammatory breast cancer

Paget’s disease of the nipple

Is this new?

No, known for more than 100 years…

What is it?

A special clinical manifestation of very aggressive breast cancer.

A special clinical manifestation of a very slow-growing, indolent breast cancer.

What is the time-frame?

The history is weeks only.

The history is months and at times years.

How often does it occur?

Only about 1- 2% of all breast cancers in our breast clinic are true inflammatory breast cancers – that’s why it is often missed.

Less than 1% of all breast cancers in our breast clinic manifest as Paget’s disease of the nipple – that’s why it is often missed.

How do I recognise it?

An area of redness of the skin of the breast that enlarges in the absence of fever or other signs of infection – this can be a rash that comes and goes, but is localised to the skin on the breast area. The clinical picture is very distinctive and an experienced doctor with a special interest in breast health will recognise it with one glance.

A small scab or erosion of the nipple that very slowly enlarges and is present only on one nipple. There is no redness associated. The clinical picture is very distinctive and an experienced doctor with a special interest in breast health will recognise it with one glance.

How is the diagnosis made?

A skin biopsy shows characteristic invasion of lymph vessels by cancer – mammography and ultrasound are usually not helpful.

A biopsy of the nipple shows an early cancer in the milk ducts. Mammography may show signs of an early stage, non-invasive cancer but is at times not helpful.

How is it treated?

It is imperative to start with chemotherapy; only when chemotherapy has cleared the signs of inflammation, surgery can be performed as otherwise the risk of recurrence of the cancer in the wound is very high. After surgery, radiotherapy is mandatory to decrease the chance for recurrence of the cancer. Surgery consists invariably of a mastectomy and we do not recommend reconstruction.

Surgery is the mainstay of the treatment and – for pure Paget’s disease - curative. A mastectomy with immediate reconstruction or breast conserving therapy has good results.

What is the prognosis?

Only about 30 of 100 patients are alive after 5 years with maximal therapy.

For pure Paget’s disease, about 98 of a 100 women are alive and well 10 years after the diagnosis.

Take home message:

Easily recognised by experienced breast doctors and early treatment paramount.

Easily recognised by experienced breast doctors and therapy has excellent results.

Go here to ask today's guest expert any questions relating to breast health.

Source: Professor Justus Apffelstaedt (Associate Professor, University of Stellenbosch and Head: Breast Clinic, Tygerberg Hospital); Health24

(Amy Henderson, Health24, April 2009)

 

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Ask the Expert

Breast cancer expert

Dr Gudgeon qualified in Birmingham, England, in 1968. She has more than 40 years experience in oncology, and in 1994 she founded her practice, Cape Breast Care, where she treats benign and malignant breast cancers. Dr Boeddinghaus obtained her qualification at UCT Medical School in 1994 and her MRCP in London in 1998. She has worked extensively in the field of oncology and has a special interest in the hormonal management of breast cancer. She now works with Dr Gudgeon at Cape Breast Care. Read more.

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