Inflammatory Breast Cancer: Understanding and Protecting Against This Aggressive Disease

Introduction

The other day, I met a woman as she was leaving the pool area. She was warm and approachable, and I learned she had a background as an oncology nurse, having worked first at MD Anderson and later at UCSD. A few months ago, she noticed some unusual skin lesions, and while the initial tests didn’t raise any concerns, further examinations revealed she had Stage 3B inflammatory breast cancer. Fortunately, her nursing experience likely helped her catch this rare and aggressive disease early. She is now undergoing chemotherapy, receiving a standard treatment regimen that includes Adriamycin and other oncolytic drugs. This blog will discuss this disease and explore how women can protect themselves.

What is Inflammatory Breast Cancer?

Inflammatory breast cancer (IBC) is an uncommon and highly aggressive type of breast cancer, representing just 1% to 5% of all diagnosed cases. Unlike the majority of breast cancers that usually appear as a noticeable lump, IBC tends to present with more subtle and less typical symptoms. Unlike most breast cancers that typically present as a distinct lump, IBC manifests in a different and more subtle manner. This cancer is characterized by the breast becoming red, swollen, and warm, giving it an inflamed appearance. One of the most distinguishing characteristics is the rapid onset of symptoms that often resemble an infection or inflammation rather than a typical tumor. The skin over the breast may take on a red or purplish hue, often appearing thickened or ridged, a condition known as peau d’orange, which refers to its similarity to the texture of an orange peel. This inflammatory look is caused by cancer cells blocking the lymphatic vessels in the skin of the breast, leading to fluid build-up and tissue changes. Additionally, the affected breast may feel warm to the touch, and there can be an overall sensation of heaviness. Unlike more common breast cancers, there is usually no well-defined mass detectable through physical examination or mammography. This absence of a distinct lump can delay diagnosis, as the symptoms may initially be mistaken for less serious conditions like mastitis, especially in younger women or those who are breastfeeding.

Because of its aggressive nature, the condition progresses quickly, often spreading to nearby lymph nodes or other areas of the body early in the disease course. (Image is courtesy of MD Anderson). This makes early detection and treatment particularly crucial. Treatments generally involve a combination of therapies, such as chemotherapy, surgery, radiation, and targeted treatments, aimed at addressing both the local manifestations and potential systemic spread.

Symptoms and Diagnosis

The symptoms of IBC can develop rapidly, often within three to six months. Common signs include:

The symptoms of IBC can develop very rapidly, often within a few weeks to a few months, and are generally more severe than those of other breast cancers. Common signs and symptoms of IBC include:

• Swelling (edema): The breast may appear larger or swollen due to the accumulation of fluid.
• Redness: The breast may turn red or purplish, with discoloration covering more than one-third of the breast.
• Skin Texture Changes: The skin on the breast may thicken or develop ridges, resembling an orange peel (a condition known as peau d’orange).
• Nipple Changes: The nipple may become retracted or inverted, pulling inward rather than pointing outward.
• Size Discrepancy: One breast may appear noticeably larger than the other.
• Tenderness or Pain: The affected breast may feel tender, painful, or itchy.
• Swollen Lymph Nodes: Lymph nodes under the arm or near the collarbone may become swollen and noticeable.

Because these symptoms can mimic those of a breast infection (mastitis) or other inflammatory conditions, IBC is often misdiagnosed initially. However, if symptoms persist after a course of antibiotics, it is crucial to seek further evaluation. Diagnostic tests such as a biopsy, mammogram, or MRI are necessary to confirm the presence of IBC.

Stages of Inflammatory Breast Cancer

IBC is typically diagnosed at a locally advanced stage (Stage III) or has already metastasized to distant parts of the body (Stage IV) at the time of diagnosis. Stage 3B, as in the case of the woman I met, means the cancer has spread to the skin of the breast or the chest wall. This stage requires aggressive treatment to manage the disease.

Treatment Options

Treatment for IBC usually involves a combination of therapies to address the aggressive nature of the disease. The standard treatment regimen includes:

1. Chemotherapy: Frequently used as the initial treatment to reduce the size of the tumor before surgical intervention. A widely utilized chemotherapy drug in this approach is Adriamycin (doxorubicin).
2. Surgery: After chemotherapy, a mastectomy (removal of the entire breast) is typically performed to remove as much of the cancer as possible. This may also include the removal of nearby lymph nodes.
3. Radiation Therapy: Post-surgery, radiation therapy is used to target any remaining cancer cells in the chest wall and surrounding areas.
4. Targeted Therapy: For cancers that are HER2-positive, targeted drugs such as trastuzumab (Herceptin) may be used in conjunction with chemotherapy.
5. Hormone Therapy: If the cancer is hormone receptor-positive, hormone-blocking treatments may be used to prevent the cancer from growing.

Prognosis and Survival Rates

The prognosis for IBC is generally poorer compared to other types of breast cancer due to its aggressive nature and the likelihood of being diagnosed at an advanced stage. Early detection and proactive treatment can significantly enhance outcomes. For individuals diagnosed with Stage 3 breast cancer, the five-year survival rate is around 86%.

How Women Can Protect Themselves

While there is no guaranteed way to prevent IBC, women can take several steps to reduce their risk and catch the disease early:

1. Regular Breast Self-Exams: Women should perform monthly breast self-exams to check for any unusual changes in their breasts. This includes looking for redness, swelling, or changes in skin texture.
2. Routine Screenings: Regular mammograms and clinical breast exams are essential for early detection of breast cancer. Women should follow their healthcare provider’s recommendations for screening based on their age and risk factors.
3. Healthy Lifestyle: Maintaining a healthy weight, staying physically active, and limiting alcohol consumption can help reduce the risk of breast cancer.
4. Awareness of Symptoms: Being aware of the symptoms of IBC and seeking medical attention promptly if any changes are noticed can lead to earlier diagnosis and treatment.
5. Genetic Counseling and Testing: Women with a family history of breast cancer may benefit from genetic counseling and testing to determine their risk and take preventive measures.

Conclusion

Inflammatory breast cancer (IBC) is a rare yet highly aggressive form of breast cancer, accounting for only 1–5% of cases. Despite its rarity, IBC progresses rapidly and tends to spread quickly to lymph nodes and other tissues, often leading to advanced-stage diagnosis. The aggressive nature of IBC means that prompt and intensive treatment is critical for managing the disease effectively. Unlike other types of breast cancer, IBC does not typically present with a distinct lump; instead, it manifests with symptoms like redness, swelling, and skin thickening, which can resemble a breast infection. These atypical symptoms often lead to delayed diagnosis, and by the time it is recognized, the cancer is frequently in advanced stages (Stage III or IV). Early detection is crucial, yet challenging, due to the non-lump presentation of IBC.

Recognizing symptoms like warmth, swelling, redness, and skin dimpling (resembling an orange peel) can make a significant difference in outcomes, as starting treatment quickly may improve survival rates and quality of life. Treatment for IBC is typically aggressive, involving a combination of chemotherapy, surgery, radiation therapy, and targeted treatments, all necessary to control the disease’s spread. Understanding the risk factors, such as being female, of African descent, premenopausal, or obese, along with staying informed about the disease’s unique symptoms, allows individuals to take proactive steps. Vigilance and awareness of personal risk factors, combined with early symptom recognition, enable women to seek medical evaluation quickly, which can improve the chance of a successful outcome. While prevention is not guaranteed, staying informed and taking active steps, such as regular self-exams, maintaining a healthy lifestyle, and engaging in routine medical consultations, can make a substantial difference. Inflammatory breast cancer requires immediate attention, and by staying alert to its signs, women can protect themselves, ensuring better management and a greater chance of successful treatment.

Written By: Lawrence D. Jones, Ph.D.

Keywords: IBC, inflammatory breast cancer, redness, swelling of skin, skin thickening