The rarest breast cancers may not be well-known, but for the women diagnosed with them, awareness and understanding can make a world of difference. Unlike more common breast cancer types, these rare forms often have vague symptoms and fewer treatment guidelines. That’s why it's so important to shine a light on them. Knowing what to look for can empower earlier detection and better support.
In the lead up to Breast Cancer Awareness month, BYY’s experts are bringing you all the latest info on breast cancer so you can empower yourself. By Dr Justus Apffelstaedt, specialist surgeon with an interest in breast, thyroid, and parathyroid health as well as soft tissue surgical oncology.
The World Health Organization (WHO) recently dubbed cancer a global health pandemic and local breast cancer statistics are testament to that. According to the Cancer Association of South Africa, breast cancer accounts for over 27% of all new cancer cases of women in sub-Saharan Africa[1].
“Breast cancer accounts for over 27% of all new cancer cases of women in sub-Saharan Africa.”
Specialist surgeon and founder of multi-disciplinary breast health centre, Apffelstaedt, Hoosain & Associates, Dr Justus Apffelstaedt says that deaths from breast cancer are often due to late diagnosis among other factors. “Early detection and treatment of breast cancer is crucial with all forms of breast cancers. It is even more important with rare forms of breast cancers that require a certain level of expertise to be detected. For example, Inflammatory Breast Cancer and Paget’s disease of the nipple often escape mammographic and ultrasound detection but are obvious to the skilled and experienced examiner.”
Inflammatory breast cancer is very aggressive disease and usually progresses rapidly in a matter of weeks or months, while Paget’s disease of the nipple is the manifestation of a very slow-growing, indolent breast cancer which can take months and even years to develop.
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Inflammatory breast cancer
Considered a very rare breast cancer, the earliest descriptions of this type of breast cancer were recorded over 100 years ago. Dr Apffelstaedt says only about 1-2 % of breast cancers diagnosed at his practice are true inflammatory breast cancer. It is often only detectable on mammography late in the course of the disease when the chance for cure is unfortunately minimal.
“If diagnosis and intervention are made earlier, than prognosis is more hopeful.”
Symptoms: An area of redness of the skin of the breast that enlarges in the absence of fever or other signs of infection may be an early sign of inflammatory breast cancer. “This can be a rash that comes and goes, but is localised to the skin on the breast area.
With these subtle clinical signs, it is often mistaken for an intercurrent infection, treated as such and missed; the diagnosis is therefore often delayed for crucial months. However, the clinical picture is very distinctive to an experienced doctor with a special interest in breast health who will recognise it with one glance,” says Dr Apffelstaedt.
A skin biopsy is required to properly diagnose inflammatory breast cancer as mammography and ultrasound are usually not helpful. Dr Apffelstaedt recommends chemotherapy as a start for treatment: “Only when chemotherapy has cleared the signs of inflammation, can surgery 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 reconstruction can take place if appropriate,” he explains.
And the prognosis? Unfortunately, if there has been a delay in diagnosis, with the above-mentioned treatment, only about 30 of 100 patients are alive after 5 years with maximal therapy. If diagnosis and intervention are made earlier, than prognosis is more hopeful.
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Paget’s disease of the nipple
According to Dr Apffelstaedt, this type of breast cancer causes little to no pain and grows at a very slow pace, which is why the diagnosis is often missed, sometimes even for years. “It so rare that only 1% of breast cancers diagnosed at my practice manifest as Paget’s disease of the nipple,” he says.
Symptoms: A small scab or erosion of the nipple that very slowly enlarges and is present only on one nipple can often be a sign of Paget’s disease of the nipple. There is no redness or other skin discolouration associated with this breast cancer. 
“Again, the clinical picture of Paget’s disease of the nipple is very distinctive and an experienced doctor with a special interest in breast health will recognise it instantly. For diagnosis, a biopsy of the nipple can reveal the cancer in its early stages. While mammography may show signs of an early stage, non-invasive cancer, often it is not helpful,” says Dr Apffelstaedt.
Mastectomy with immediate reconstruction or breast conserving therapy is the mainstay of the treatment for this breast cancer and about 98 out of 100 diagnosed women are alive and well 10 years after the diagnosis.
“An experienced doctor with a special interest in breast health will recognise it instantly.”
“We all know that screening saves lives, but in cases of rare forms of breast cancer, mammography is not enough and a thorough physical examination by a seasoned practitioner with an interest in breast health can be lifesaving as these cancers are easily missed.” concludes Apffelstaedt.
https://cansa.org.za/breast-cancer/
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What are the two rarest types of breast cancer discussed?
The two rare forms of breast cancer often highlighted are Inflammatory Breast Cancer (IBC) and Paget’s Disease of the Nipple. Both are uncommon and present differently from the more typical forms of breast cancer.
What is Inflammatory Breast Cancer (IBC)?
IBC is an aggressive and rare type of breast cancer that causes the breast to become red, swollen, and warm. It doesn’t always form a lump, which can delay diagnosis. It’s often mistaken for an infection initially.
What are the signs of Inflammatory Breast Cancer?
Common signs include:
- Rapid breast swelling
- Skin that looks like orange peel
- Redness and warmth in the breast
- Flattening or inversion of the nipple
These symptoms usually develop quickly over weeks.
What is Paget’s Disease of the Nipple?
Paget’s Disease affects the skin of the nipple and areola and is usually linked to an underlying breast cancer. It resembles eczema but does not respond to typical topical treatments.
What are the symptoms of Paget’s Disease?
Look out for:
- Itching, redness or flaking of the nipple skin
- Yellowish or bloody discharge
- A lump in the breast (in some cases)
- Burning or tingling sensations
How are these rare breast cancers diagnosed?
Diagnosis often involves:
- A physical exam
- Mammography or ultrasound
- Biopsy of affected tissue
- MRI for further evaluation
Due to their unusual presentation, early detection can be more challenging.
Are these cancers treatable?
Yes. Both IBC and Paget’s Disease can be treated, often involving a combination of surgery, chemotherapy, radiation, and hormone therapy depending on the stage and spread.
How common are these cancers in South Africa?
While there is limited local data due to underreporting and misdiagnosis, both types remain rare worldwide. IBC accounts for 1–5% of all breast cancers, and Paget’s Disease is even less common.
Can men develop these rare breast cancers?
Yes. Although rare in men, Paget’s Disease and IBC can still occur. Any unusual changes in the nipple or breast area in men should be investigated.
When should I see a doctor?
Seek medical advice if you notice rapid changes in your breast, persistent skin irritation, discharge from the nipple, or any unusual lump or swelling. Early medical attention improves outcomes.
Is there support available in South Africa?
Yes. Organisations such as CANSA and PinkDrive offer support, education, and access to screening. Government hospitals and clinics also offer breast health services, including referrals to oncology units.\
Disclaimer: This FAQ is for informational purposes only and not a substitute for professional medical advice. Always consult a qualified healthcare provider for diagnosis and treatment options.
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