Rare breast cancer very dangerous

Most people associate breast cancer with lumps in the breast, but there is another very dangerous form that disguises itself as an infection, often leading to delayed diagnosis.

Inflammatory breast cancer is a rare, but important form of breast cancer. Only about 1% of all breast cancers are inflammatory.

Its hallmark is redness and swelling of the skin, which closely mimics a breast infection. This appearance confuses us into believing that we’re dealing with a relatively harmless infection.

Inflammatory breast cancer survivor Louise Bertoni (58) from Goodwood in Cape Town tells of her experience. "At first the left breast was red and warm," explains Louise. She went to her GP who treated her for mastitis (breast infection) and the condition cleared up. "But one month later the right breast also became red and swollen, so my GP sent me for a mammogram immediately."

This picture of inflammation is due to the early spread of this cancer to the lymph vessels beneath the skin. This has two characteristic results: tumour cells block these lymph vessels, leading to swelling of the skin with the characteristic “orange peel” appearance; and it can cause an intense inflammatory reaction in the lymph vessels, hence the red discoloration of the skin, which at times can even deepen to a purple or blue tinge.

In the early stages, the redness may resemble an insect bite and may shift over the course of a few days or a week. Antibiotics are often prescribed, but obviously have no effect.

A give-away that it’s not a standard infection, is that the patient rarely has pain and almost never a fever, as would be expected with an infection.

Furthermore, due to the early access that the tumour gains to the lymphatic system, the underarm glands are often painlessly enlarged, and hard. Confusingly, often no mass can be felt in the breast or seen in the imaging examinations.

A mammogram, biopsy, ultrasound, bone scan and a chest X-ray confirmed that Louise had inflammatory breast cancer. "I first cried at the thought that I could be dying," she says. "But after about three days I realised that cancer is not a death sentence and that I must be positive."

This diagnosis didn't just affect Louise. "My husband was devastated at first. But he was very supportive and took over at home. He couldn't do enough for me."

Diagnosis is often missed and effective treatment becomes more difficult. Making the correct diagnosis requires suspicion and a great deal of experience. Needle core biopsies of the breast or fine needle aspiration of the lymph nodes might be requested; as well as skin biopsies to demonstrate tumour cells in the lymph vessels under the skin.

Treatment starts with aggressive chemotherapy, aimed at “cleaning out” lymph vessels. This is necessary otherwise, during surgery, cancer cells could pour out of the lymph vessels into the surgical wound, leading to recurrence of the cancer within a couple of months.

"The first (chemotherapy) session was emotional," Louise confesses. "My husband and friends supported me all the way. They sat with me during my chemo treatment, brought books to read during the four hours, and even packed picnic baskets."

Louise's cancer was very aggressive and they decided to have a mastectomy (breast removal) before it spread any further.

Following chemotherapy, a mastectomy might be performed. With the mastectomy, usually all the underarm glands are removed too. To conclude the treatment regimen, the chest wall is irradiated.

Hormonal therapy is only rarely indicated, as – in keeping with the aggressive, fast growth of these tumours – they are usually not hormone dependent.

Radiation takes five to seven weeks of daily sessions, and the chest wall, the border of the neck and the armpit and the areas to the left and the right of the sternum are targeted as these cover the most common pathways of lymphatic spread.

"Four weeks after my mastectomy I underwent 26 radiations," tells Louise. "That made me very tired." Still, her treatment went well and soon she was back on her feet again.

In contrast with other forms of breast cancer, biologicals in inflammatory breast cancer are given before surgery. Even after this aggressive treatment, the chance of local recurrence on the chest wall and in distant organs such as the lungs, liver or bones remains relatively high in comparison to more common types of cancer.

Five years after the diagnosis, only about 50% of patients are still alive and 20 years after the diagnosis, only about a third of patients survive.

It is now a year since Louise's initial diagnosis and she is doing well. Her latest check-up showed that she's still free of cancer and all treatment, including chemotherapy, has been ceased.

Louis says she's adopted a more healthy lifestyle and that she doesn't stress about little things so much anymore.

It has even changed her relationships with friends and family. "My relationship with my husband is warmer," and she has also grown closer to her family and her maker.

She advises women to do regular self-examinations, go for mammograms and to enjoy the good things in life. "You have to be positive, it's not a death sentence. Live life to the full and remember God is in control of your life."

- (Wilma Stassen and Professor Justus Apffelstaedt, associate professor, University of Stellenbosch and head of the Breast Clinic, Tygerberg Hospital, Health24)

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