It is referred to as "inflammatory" due to its frequent presentation with symptoms resembling a skin inflammation, such as erysipelas.
[3] Typical presentation is rapid breast swelling, sometimes associated with skin changes (peau d'orange), and nipple retraction.
[5] Recent advances in therapy have improved the prognosis considerably; at least one-third of women will survive with IBC for ten years or longer.
Rapid onset of symptoms is typical; the breast often looks swollen and red, or "inflamed", sometimes seemingly changing overnight.
Temporary regression or fluctuation of symptoms, spontaneously or in response to medications or hormonal events should not be considered of any significance in diagnosis.
A large number of IBC cases present as triple negative breast cancer (TNBC).
Searches for biomolecular characteristics has produced a broad range of possible biomarkers, such as loss of LIBC and WISP3 expression.
[citation needed] Estrogen and progesterone receptor status is frequently negative, corresponding with poor survival.
A number of proteins and signalling pathways show behaviour of biochemicals which can be considered paradoxical, compared with their function in normal tissue as well as in other breast cancer types.
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.
[citation needed] Staging is designed to help organize the different treatment plans and to understand the prognosis better.
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.
[23] Loss of diploidy (heterozygosity) and extensive breast inflammation upon first clinical examination are associated with a significantly worse IBC prognosis.