Воспалительный рак молочной железы: симптомы, диагностика и лечение.

Inflammatory breast cancer (IBC) is one of the most aggressive types of breast cancer. It can occur in women of any age (and, extremely rarely, in men, see male breast cancer). It is referred to as "inflammatory" due to its frequent presentation with symptoms resembling a skin inflammation, such as erysipelas. Inflammatory breast cancer presents with variable signs and symptoms, frequently without detectable lumps or tumors; it therefore is often not detected by mammography or ultrasound. Typical presentation is rapid breast swelling, sometimes associated with skin changes (peau d'orange), and nipple retraction. Other signs include redness, persistent itching, and unusually warm skin. IBC often initially resembles mastitis. Approximately 50% to 75% of cases have the typical presentation; an atypical presentation makes diagnosis more difficult. In some cases, a sign such as acute central venous thrombosis may be the sole presenting indication of the disease. IBC comprises a small proportion of breast cancer cases (1% to 6% in the USA). African Americans are usually diagnosed with IBC at younger ages than Caucasian women, and they are also at higher risk for the disease. Recent advances in therapy have improved the prognosis considerably; at least one third of women will survive with IBC for ten years or longer.

The reliable method of diagnosis by imaging, Mammography, breast MRI or ultrasound, which often show suspicious signs (general skin edema, skin thickening, mass, suspected breast lesions). It is important to biopsy the suspected lesions and/or skin. However, despite significant effort, a diagnosis could be missed. Therefore, repeat imaging and biopsies are important if a diagnosis of IBC is suspected. Clinical presentation is typical in only 50% to 75% of cases; many other conditions, such as mastitis or even cardiac insufficiency, can mimic the typical symptoms of inflammatory breast cancer. Temporary regression or fluctuation of symptoms, spontaneously or in response to medications or hormonal events should not be considered of any significance in diagnosis. Treatment with antibiotics or progesterone have been observed to cause a temporary regression of symptoms in certain cases.

IBC occurs in all adult age groups. While the majority of patients are between 40 and 59 years old, age predilection is much less pronounced than in noninflammatory breast cancer. The overall rate is 1.3 cases per 100000; black women (1.6) have the highest rate, Asian and Pacific Islander women the lowest (0.7) rates. Whether inflammation contributes to the development of this disease remains an area of ongoing research.

Age distribution and relation to breastfeeding duration is suggestive of the involvement of hormones in the causation of IBC; however, significant differences exist between IBC and other breast cancers. Typically, IBC shows low levels of estrogen and progesterone receptor sensitivity, which corresponds with poor outcome. In IBC cases with positive estrogen receptor status, antihormonal treatment is believed to improve outcome. Paradoxically, some findings suggest that especially aggressive phenotypes of IBC are characterised by a high level of NF kappaB target gene expression, which can be, under laboratory conditions, successfully modulated by estrogen, but not by tamoxifen.

Staging is designed to help organize the different treatment plans and to understand the prognosis better. Staging for IBC has been adapted to meet the specific characteristics of the disease. IBC is typically diagnosed in one of these stages:
Stage IIIB at least 1/3 of the skin of the breast is affected, and cancer may have spread to tissues near the breast, such as the chest skin or chest wall, including the ribs and muscles in the chest. The cancer may have spread to lymph nodes near the breast or under the arm. Stage IIIC N3 nodal involvement with an inflamed breast will upgrade the disease from Stage IIIB to Stage IIIC. Stage IV means that the cancer has spread to other organs. These can include the bones, lungs, liver, and/or brain.

The standard treatment for newly diagnosed inflammatory breast cancer is to receive systemic therapy prior to surgery, followed by the radiation therapy. Achieving "no disease [pathological complete response (pCR)]" in the surgical samples gives the best prognosis. Surgery is modified radical mastectomy. Lumpectomy, segmentectomy, or skin sparing mastectomy are not recommended. Immediate reconstructive surgery is not recommended. Immediate,"upfront" surgery is contraindicated, as results are better using neoadjuvant chemotherapy first. Contralateral prophylactic mastectomy is not recommended because it can delay the other systemic adjuvant treatment or adjuvant radiation therapy. After surgery, all cases are recommended for radiation therapy unless it is contraindicated. Due to the aggressive nature of the disease, it is highly recommended that people with IBC be seen by an IBC specialist and by a multidisciplinary team of health workers. Exploring whether clinical trials are available is very important. In patients with newly diagnosed IBC with metastatic diseases, it is essential to discuss whether palliative surgery of the breast is indicated after the systemic treatment. In the non IBC setting, palliative surgery is not recommended; however, for IBC, palliative surgery to improve the QOL and to improve the long term outcome is explored in certain medical conditions. It is critical for people with IBC to seek novel targeted therapy in a clinical trial setting. Three modality combination therapy: surgery, chemotherapy, and radiation, was, in 2014, reported as being under utilized in the USA. Estrogen and progesterone receptor positive cases of IBC have not been shown to have a better prognosis than hormone receptor negative cases. Pathological complete response to preoperative chemotherapy imparts a more favorable prognosis than a pathological complete response to surgery. Loss of diploidy (heterozygosity) and extensive breast inflammation upon first clinical examination are associated with a significantly worse IBC prognosis. A premenopausal occurrence of IBC has a significantly worse prognosis than a postmenopausal diagnosis. In postmenopausal cases, lean women have a significantly better prognosis than obese women. Among breast cancer patients with distant metastasis at diagnosis (stage IV disease), the overall survival (OS) is worse in patients with IBC than in those with non IBC breast cancers.