Compared with breast cancer in general, inflammatory breast cancer (IBC) is much rarer and is extremely aggressive.1
In IBC, cancer cells obstruct lymphatic vessels in the breast, causing erythema and edema. This article discusses the signs and symptoms of IBC, as well as its risk factors and treatment options.
Only 1% to 5% of new breast cancer cases in the United States are classified as IBC.2–5 Compared with other types of breast cancer, IBC is often diagnosed in younger women; the mean age for IBC diagnosis is 58.8 years, compared with 66.2 for locally advanced breast cancer.4
And because IBC is likely to have metastasized at the time of diagnosis, it is characterized by a lower survival rate compared with that of non-IBC breast cancer.2,6
IBC's dismal five-year survival rate of only 25% to 50% has remained unchanged for more than 30 years1,4—in stark contrast to the average survival rate for all breast cancer types of 75%, and for early-stage, non-IBC of 90%.4 The median survival time for patients diagnosed with IBC is only 37 months.5
Race, ethnicity, and gender also appear to play a role in IBC (see “Other Factors” box).
Signs & Symptoms Can Be Misleading
The American Joint Committee on Cancer describes the appearance of IBC as “diffuse erythema and edema of the breast that arises quickly, often without an underlying palpable mass.”5 According to a panel of leading IBC experts, the minimum clinical diagnostic criteria for IBC include these four points:2
- Rapid onset of breast erythema, edema, and/or peau d'orange (resembling the skin of a navel orange, swollen with dimples), and/or a warm breast, with or without an underlying palpable mass.3
- Duration of history of six months or less.
- Erythema involving at least one-third of the breast.
- Pathologic confirmation of invasive cancer.2,4,7
Reaching a Diagnosis
The clinical exam, which is the primary means of diagnosis for IBC, is followed by core breast biopsy and imaging studies to confirm the diagnosis.1 Patients meeting the diagnostic criteria for IBC are strongly recommended to have at least two skin-punch biopsies to determine the presence of dermal lymphatic invasion, which is considered to be a histologic hallmark of IBC.2
The optimal site for the biopsies is the area of the affected breast with the most prominent skin discoloration.2 Any malignant tissue identified through biopsy is subjected to further testing. Hormone-receptor and HER2-receptor tests are performed and the results are used to design a treatment regimen for the patient.
Hormone-receptor status determines whether the cancer cells contain estrogen and/or progesterone receptors on the surface of the cell and whether the cancer cells need high estrogen or progesterone levels to grow—i.e., are ER+ or PR+.3,8
Hormone receptor-positive cells respond more favorably to hormone therapy, and these patients tend to have a better prognosis.8
Although two of three breast cancers have at least one of these hormone receptors, most IBCs are hormone-receptor negative.2,3,8,9 Because hormone receptor-negative cells are less likely to respond to hormone therapy, they are associated with a more aggressive disease process, and women with IBC tumors have a lower overall and breast cancer-specific survival rate.2
HER2 status determines whether the cancer cells contain human epidermal growth factor receptor-2. Cells that test positive for the HER2 receptor attract too many growth factors, contributing to the cancer's rapid growth.3 Overexpression of the HER2 protein is found twice as often in IBC tumors compared with noninflammatory tumors.9 Because receptor status can help determine the prognosis and guide therapy, the recommendation is for all IBC tumors to be tested for hormone and HER2 receptors.2,7
Imaging studies, such as diagnostic mammography, ultrasound, and magnetic resonance imaging (MRI), help the clinician define the area for biopsy and determine the presence or absence of metastasis.2
- Diagnostic mammography. IBC manifests as sheets, also called nests, within the breast and can mask any masses or calcifications, making mammography alone an ineffective tool for detecting and staging IBC.3,4 Mammography, however, is useful in detecting the skin thickening and trabecular distortion that often occurs with IBC. All women with suspected IBC should have a diagnostic mammogram with an accompanying ultrasound.2,3,9
- Ultrasound. A panel of leading IBC experts has recommended that an ultrasound of the breast and regional lymph nodes accompany the diagnostic mammogram.2 Ultrasound of the breast and regional lymph nodes is particularly useful in pinpointing areas for potential biopsy, such as underlying masses, parenchymal architectural distortions, and involved regional lymph nodes.2,4
- MRI. Although MRI is a popular diagnostic imaging tool, data on its use in IBC are scarce.2 Breast MRI has several drawbacks, including high cost, a greater time commitment, and limited sizes of breast coils. Until more data are available, routine diagnostic MRI is not recommended.2 The IBC panel does recommend the use of diagnostic MRI, however, in cases where the breast parenchymal lesions are not detected by mammogram or ultrasound and in clinical trials studying the use of MRI in IBC.2
Because of the aggressiveness of IBC, almost 35% of patients with IBC have early disease metastasis, commonly to the bone, liver, and lungs.4,5 Additional imaging tools such as chest radiographs, computed tomography, and bone scans can be used to document the extent of metastasis.2,4,7
Positron emission tomography-computed tomography is being studied for use early in the disease state to help determine the extent of metastasis, map any tumors, and guide and evaluate treatment.4
At the time of diagnosis, IBC is staged as IIIB, IIIC, or IV.1–3,7
The preferred treatment regimen for IBC is a combined-modality regimen beginning with chemotherapy, followed by mastectomy and radiation therapy.2,4,5 Because widespread metastasis is likely, breast-conservation surgery is usually not an option.4
The current recommendation is generally that all IBC patients receive anthracyclines, such as doxorubicin or epirubicin, in combination with taxanes, such as paclitaxel or docetaxel.2,3
Patients who test positive for hormone receptors should have hormone therapy designed to block the hormone receptors and prevent estrogen and progesterone from attaching to the cancer cells. Drugs used for these purposes include tamoxifen and trastuzumab.3 Patients with positive HER2-receptors will likely receive targeted therapy using trastuzumab in combination with the primary systemic chemotherapy.2,3 Leading IBC experts recommend that patients undergo a minimum of six chemotherapy cycles over four to six months before continuing to definitive surgery.2 This plan of treatment may be changed if IBC progression is detected.2
After chemotherapy, localized treatments, such as surgery and chest wall radiation, are used to eradicate the remaining cancer cells.1 Modified radical mastectomy is the recommended definitive surgery for IBC patients.2
Radiation therapy, which is most effective after mastectomy, targets the skin, chest wall, and axillary, infraclavicular, supraclavicular, and internal mammary lymph nodes.2,4,5 Although clinicians at one facility believe that 44 radiation treatments (delivered twice a day for 22 treatment days) are needed to combat the highly prolific IBC cells, the current regimen is one radiation treatment a day for 25 to 35 treatment days, spread over five to seven weeks.5,10
Breast reconstruction surgery is possible, but the radical nature of the mastectomy and skin changes resulting from radiation make successful breast reconstruction difficult. At the present time, immediate breast reconstruction is not recommended. More studies are needed to determine the effect of breast reconstruction on radiation coverage to the breast and lymph nodes.2
Follow-up: After the trimodal treatment regimen is completed, patients should have a follow-up physical exam every three to six months and have an annual mammogram of the unaffected breast.2 Additionally, genetic screening for those patients with a strong family history of breast or ovarian cancer and annual ultrasound of the locoregional lymph nodes may be conducted.2
Conditions that Mimic Inflammatory Breast Cancer
Certain conditions can mimic certain features of IBC such as mastitis or abscess, cellulitis, allergic rash, breast tenderness associated with fibrocystic breast disease, and insect or spider bites;4,5,9 in addition, fever accompanies many infections, such as mastitis and cellulitis, but not IBC.3,9
In the case of a possible breast infection, women should be advised to ask their health care provider about ruling out IBC if their condition does not respond to a one-week regimen of antibiotics.5 Treatment for mastitis or abscess can include multiple antibiotics over several weeks; during this time, undiagnosed IBC could rapidly advance.4 The current recommendation is to biopsy the area if inflammation remains after one week of antibiotic therapy.4
Supporting the Patient
A diagnosis of IBC can be devastating. At the time of diagnosis, IBC is staged as a IIIB, IIIC, or IV cancer.1–3,7 Empower patients by directing them to organizations that provide information about IBC treatments, research, and support. Also provide information about clinical trials and encourage patients to participate. The National Comprehensive Cancer Network, for example, has a website, http://www.nccn.com/clinicaltrials.aspx, to help patients find a clinical trial in their area.7
Awareness of IBC is growing. In 2006, the first clinic in the world for women with IBC was established at the University of Texas MD Anderson Cancer Center, and the first international conference on IBC was held in December 2008.4,11
International IBC-specific tumor registries and banks are currently being developed with the aim to increase knowledge of IBC epidemiology and research.2
The IBC registry, which is currently accepting registrants, can be found at http://clinicaltrials.gov/ct2/show/NCT00477100
Because of the poor prognosis for patients diagnosed with IBC, one of the most caring and compassionate actions a nurse can take is to provide supportive care, allowing the patient to enjoy the highest quality of life possible. As nurses, we save lives and we can continue to do so by increasing awareness of inflammatory breast cancer.
Adapted from Nursing 2010;40(9):58-62
Signs & Symptoms2,11
- Edema, often sudden, of one or both breasts.
- Rash or erythema in small patches or over the entire breast.
- Skin on the affected breast that resembles the skin of an orange.
- Ridges or thickened areas of the breast.
- Breast skin that is warm or hot to the touch.
- Flat or inverted nipples with changes in the color and texture of the areola.
- Nipple discharge.
- Breast pain, fullness, or pruritus.
- Lymphadenopathy in the neck and axilla.
African American women are diagnosed with inflammatory breast cancer at higher rates than white women and have a lower median survival time—two as opposed to three years.5 R6-3,6 Hispanic women tend to develop IBC at an earlier age—50 compared with 55 for African American women and 58 for white women.4 IBC affects men, too, with 1.4% of all male breast cancer patients from 1973 to 2000 being diagnosed with IBC.5
- Inflammatory Breast Cancer Research Foundation: http://www.ibcresearch.org
- National Cancer Institute: http://www.cancer.gov
- Inflammatory Breast Cancer Foundation: http://www.eraseibc.com
- National Comprehensive Cancer Network: http://www.nccn.org
- American Cancer Society: http://www.cancer.org
- Breastcancer.org: http://www.breastcancer.org
- MD Anderson Cancer Center: Inflammatory Breast Cancer: http://bit.ly/MDACCIBC