Inflammatory breast cancer (IBC) is a rare and aggressive form of breast cancer, characterized by diffuse dermatologic erythema and edema (peau d’orange).

It accounts for 0.5 to 2 percent of invasive breast cancers diagnosed in the United States, but may be higher elsewhere [1,2]. As compared with locally advanced breast cancer, IBC is diagnosed at an earlier age (a median of 59 versus 66 years of age) [2]. The incidence of IBC is higher in black Americans compared with whites, and black women are diagnosed at a younger age [1,2]. Data on risk factors are limited and inconclusive [1].

Patients with inflammatory breast cancer (IBC) typically present with breast pain or a rapidly growing, self-diagnosed breast lump [3]. They may also report a tender, firm, or enlarged breast, or itching of the breast. On presentation, almost all women with IBC have lymph node involvement, and approximately one-third have distant metastases [4,6].

IBC is designated as T4d in the American Joint Committee on Cancer (AJCC) Tumor, Node, Metastasis (TNM) staging system [7].

Criteria must be met for diagnosis of IBC [8]:

• Rapid onset of breast erythema, edema and/or peau d’orange, and/or warm breast, with or without an underlying palpable mass.
• Duration of history no more than six months.
• Erythema occupying at least one-third of the breast.
• Pathologic confirmation of invasive carcinoma.

Multimodality therapy is standard for non-metastatic disease and includes neoadjuvant chemotherapy followed by mastectomy and postmastectomy radiation.

Neoadjuvant therapy, we utilize an anthracycline- and taxane-based dose-dense chemotherapy regime. Additional therapies depend on tumor receptor status:

Patients with IBC and human epidermal growth factor receptor 2 (HER2) overexpression should receive HER2-directed therapy (trastuzumab with pertuzumab) with neoadjuvant chemotherapy, although we avoid giving trastuzumab and anthracycline concurrently given the risk for cardiotoxicity. Trastuzumab with or without pertuzumab should be continued postoperatively for a total of one year of treatment if Pathologic Complete Response (PCR) is achieved. Otherwise if we do not receive PCR, adjuvant therapy will be 14 cycles with kadcyla.

For women with hormone receptor-positive disease, endocrine therapy should be initiated after completion of neoadjuvant therapy and continued in the adjuvant setting.

For patients with non-metastatic disease for whom surgery is not feasible even after the completion of a course of neoadjuvant chemotherapy, further efforts are directed at downstaging the tumor such that surgery may be performed. While data in this setting are limited, our approach is to utilize single-agent chemotherapies such as carboplatin, vinorelbine, or capecitabine for patients who are candidates for further chemotherapy. If patients are not candidates for further chemotherapy, or if the tumor continues to be chemoresistant after two or three lines of systemic treatment, we next utilize RT in attempts to decrease the extent of the disease. For patients whose tumor becomes resectable with these treatments, mastectomy and axillary node dissection should be pursued.

1. Hance KW, Anderson WF, Devesa SS, et al. Trends in inflammatory breast carcinoma incidence and survival: the surveillance, epidemiology, and end results program at the National Cancer Institute. J Natl Cancer Inst 2005; 97:966.
2. Anderson WF, Schairer C, Chen BE, et al. Epidemiology of inflammatory breast cancer (IBC). Breast Dis 2005-2006; 22:9.
3. Matro JM, Li T, Cristofanilli M, et al. Inflammatory breast cancer management in the national comprehensive cancer network: the disease, recurrence pattern, and outcome. Clin Breast Cancer 2015; 15:1.
4. Kleer CG, van Golen KL, Merajver SD. Molecular biology of breast cancer metastasis. Inflammatory breast cancer: clinical syndrome and molecular determinants. Breast Cancer Res 2000; 2:423.
5. Jaiyesimi IA, Buzdar AU, Hortobagyi G. Inflammatory breast cancer: a review. J Clin Oncol 1992; 10:1014.
6. Walshe JM, Swain SM. Clinical aspects of inflammatory breast cancer. Breast Dis 2005-2006; 22:35.
7. In: American Joint Committee on Cancer Staging Manual, Eighth edition, Amin, Mahul (Eds), 2017.
8. Dawood S, Merajver SD, Viens P, et al. International expert panel on inflammatory breast cancer: consensus statement for standardized diagnosis and treatment. Ann Oncol 2011; 22:515.

STYLIANOS GIASSAS
MEDICAL ONCOLOGIST
CHIEF OF THE 2 ONCOLOGY CLINIC IASO HOSPITAL