Inflammatory breast cancer: Difference between revisions
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==Diagnosis== |
==Diagnosis== |
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Only reliable method of diagnosis is [[biopsy]]. [[Mammography]], [[MRI]] or ultrasound often show suspicious signs however in a significant proportion of cases they would miss a diagnosis. |
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Standard diagnostic tests for breast cancer, such as mammograms, MRI and biopsies, generally cannot accurately diagnose IBC. The following tests are used to make a diagnosis: |
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Clinical presentation is typical only in 50-75% of cases, on the other hand any number of conditions such as [[mastitis]] or even heart insufficiency can mimic the typical symptoms of IBC. |
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*Surgical biopsy: larger samples of the breast skin and underlying tissue can be collected in a surgical or skin biopsy, with better chances for identifying the cancer cells. |
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*[[Positron emission tomography|PET]] scan: In the near future, this could be one of the most important diagnostic/staging tests for IBC, though it is still under study. PET scans enable oncologists to see more disease. |
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Temporary regression or fluctuation of symptoms, spontaneous or in response to conventional treatment or hormonal events should not be considered of any significance in diagnosis. Inflammatory breast cancer is known to respond to antibiotics, progesterone and reaction to other medications or hormones can not be excluded. <ref>{{cite pmid|8085857}}</ref><ref>{{cite pmid|1387777}}</ref><ref>{{cite pmid|16740744}}</ref><ref>{{cite pmid|17700572}}</ref><ref>{{cite pmid|9238713}}</ref> |
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Inflammatory breast cancer is distinguished from other [[carcinoma]]s by [[physical exam]] showing [[inflammation|inflamed]] appearance of the affected breast.<ref name=Hortobagyi_2003 /> |
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==Characterization== |
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Inflammatory breast cancer is high grade [[aneuploid]] cancer, with mutations and overexpression of p53, high levels of E-cadherin and abnormal cadherin function. It is often regarded as a systemic cancer. |
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It is characterised by the presence of cancer cells in the subdermal lymphatics on skin biopsy. |
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Search for biomolecular characteristics produced a broad number of possible markers, such as loss of LIBC and WISP3 expression. Inflammatory breast cancer is in many ways very similar to late stage or metastatic breast cancer however it can be distinguished from those cancer types both by molecular footprint and clinical presentation. On molecular level some similarity exists with pancreatic cancer. |
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Estrogen and Progesterone receptor status is frequently negative, corresponding with poor survival. The tumors are highly angiogenic and vascular, with high levels of [[VEGF]] and [[bFGF]] expression. |
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A number of proteins and signalling pathways show behaviour that can be considered paradoxical compared to their function in normal tissue and other breast cancer types. |
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* caveolin-1 and -2 are overexpressed and may contribute to tumour tumour cell motility<ref>{{cite pmid|16244790}}</ref> |
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* E-cadherin is overexpressed and paradoxically associated with especially aggressive type. |
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RhoC GTPase is overexpressed, possibly related with overexpression (hypomethylation) of Caveolin-1,-2. Caevolin is paradoxically tumour promoting. NF-kappaB pathway activation overexpression may contribute to the inflammatory phenotype. |
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==Epidemiology== |
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Occurs in all adult age groups. While the majority of patients are between 40-59 years old, age predilection is much less pronounced than in noninflammatory breast cancer. 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.<ref>{{cite pmid|15090727}}</ref> |
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Most known breast cancer risk predictors do not apply for inflammatory breast cancer. It maybe be slightly associated with cumulative breast-feeding duration.<ref>{{cite pmid|16198566}}</ref> While there may be a slight risk correlation with breast feeding duration it must be considered that the resulting effect would be by far outweighed by the opposite correlation of breastfeeding and the more frequent breast cancer types. |
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==Role of hormones== |
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Age distribution and relation to breastfeeding duration is suggestive of some sort of involvement of hormones in the aetiology, however significant differences exist compared to normal breast cancer. |
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Typically IBC shows low levels of estrogen and progesterone receptor sensitivity corresponding with poor outcome. In cases with positive estrogen receptor status antihormonal treatment is believed to improve outcome. |
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Paradoxically some findings suggest that especially aggressive phenotypes of IBC are characterised by high level of NF kappaB target gene expression which can be - under laboratory conditions successfully modulated by estrogen but not tamoxifen. |
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==Staging== |
==Staging== |
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Revision as of 23:14, 20 August 2010
| Inflammatory breast cancer | |
|---|---|
| Specialty | Dermatology, oncology  |
Inflammatory breast cancer[1] is an especially aggressive[2] type of breast cancer that can occur in women of any age (and extremely rarely, in men).
It is called inflammatory because it frequently presents with symptoms resembling an inflammation. However it can present with very variable signs and symptoms, frequently without detectable tumors and therefore is often not detected by mammography or ultrasound.[3]
Typical presentation is rapid swelling, sometimes associated by skin changes (peau d' orange), and nipple retraction. Other symptoms include rapid increase in breast size, redness, persistent itching, skin hot to touch. IBC often initially resembles mastitis.
Only about 50-75% cases have the typical presentation. Symptoms can be completely atypical such as acute central venous thrombosis as the sole presenting symptom.
IBC only makes up 1-6% of all breast cancer cases in the USA.[4] IBC is often diagnosed in younger women although average age of presentation does not differ much from other kinds of breast cancer (average age 57 years). African-Americans are usually diagnosed at younger ages than Caucasian women, and also have a higher risk of getting IBC.[5] Recent advances in therapy have improved the prognosis considerably and at least one third of women will survive the diagnosis by 10 years or longer.[6]
Symptoms
Symptoms are very variable and may not be present at all in occult inflammatory breast cancer. Quick onset of symptoms is typical, the breast often looks swollen and red, or “inflamed”, sometimes overnight, and are easy to misdiagnose as mastitis. Invasion of the local lymphatic ducts impairs drainage and causes edematous swelling of the breast. Because the skin of the breast is tethered by the suspensory ligament of Cooper, the accumulation of fluid may cause the skin of the breast to assume a dimpled appearance similar to an orange peel (peau d'orange). IBC is sometimes misdiagnosed as an insect bite or breast infection. In the case of IBC, a lump is usually not present as in other forms of breast cancer.
Symptoms may include:
- Pain in the breast
- Skin changes on breast
- Reddened area with texture resembling the peel of an orange (peau d’orange)
- Sudden swelling of the breast
- Itching of breast
- Nipple retraction (flattened look)or discharge
- Swelling of lymph nodes under the arm or in the neck
- Unusual warmth of the affected breast
- Breast is harder or firmer
Other symptoms may rarely include:
- Swelling of the arm
- Breast decreases instead of increasing
- Although a dominant mass is present in many cases, most inflammatory cancers present as diffuse infiltration of the breast without a well-defined tumor.
- A lump may become present and grow rapidly
Most patients do not experience all the symptoms of IBC. Not all symptoms need to be present in order to be diagnosed.[7]
Diagnosis
Only reliable method of diagnosis is biopsy. Mammography, MRI or ultrasound often show suspicious signs however in a significant proportion of cases they would miss a diagnosis.
Clinical presentation is typical only in 50-75% of cases, on the other hand any number of conditions such as mastitis or even heart insufficiency can mimic the typical symptoms of IBC.
Temporary regression or fluctuation of symptoms, spontaneous or in response to conventional treatment or hormonal events should not be considered of any significance in diagnosis. Inflammatory breast cancer is known to respond to antibiotics, progesterone and reaction to other medications or hormones can not be excluded. [8][9][10][11][12]
Characterization
Inflammatory breast cancer is high grade aneuploid cancer, with mutations and overexpression of p53, high levels of E-cadherin and abnormal cadherin function. It is often regarded as a systemic cancer.
It is characterised by the presence of cancer cells in the subdermal lymphatics on skin biopsy.
Search for biomolecular characteristics produced a broad number of possible markers, such as loss of LIBC and WISP3 expression. Inflammatory breast cancer is in many ways very similar to late stage or metastatic breast cancer however it can be distinguished from those cancer types both by molecular footprint and clinical presentation. On molecular level some similarity exists with pancreatic cancer.
Estrogen and Progesterone receptor status is frequently negative, corresponding with poor survival. The tumors are highly angiogenic and vascular, with high levels of VEGF and bFGF expression.
A number of proteins and signalling pathways show behaviour that can be considered paradoxical compared to their function in normal tissue and other breast cancer types.
- caveolin-1 and -2 are overexpressed and may contribute to tumour tumour cell motility[13]
- E-cadherin is overexpressed and paradoxically associated with especially aggressive type.
RhoC GTPase is overexpressed, possibly related with overexpression (hypomethylation) of Caveolin-1,-2. Caevolin is paradoxically tumour promoting. NF-kappaB pathway activation overexpression may contribute to the inflammatory phenotype.
Epidemiology
Occurs in all adult age groups. While the majority of patients are between 40-59 years old, age predilection is much less pronounced than in noninflammatory breast cancer. 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.[14]
Most known breast cancer risk predictors do not apply for inflammatory breast cancer. It maybe be slightly associated with cumulative breast-feeding duration.[15] While there may be a slight risk correlation with breast feeding duration it must be considered that the resulting effect would be by far outweighed by the opposite correlation of breastfeeding and the more frequent breast cancer types.
Role of hormones
Age distribution and relation to breastfeeding duration is suggestive of some sort of involvement of hormones in the aetiology, however significant differences exist compared to normal breast cancer.
Typically IBC shows low levels of estrogen and progesterone receptor sensitivity corresponding with poor outcome. In 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 high level of NF kappaB target gene expression which can be - under laboratory conditions successfully modulated by estrogen but not tamoxifen.
Staging
Staging is designed to help organize the different treatment plans and to understand the prognosis better. IBC has typically this stages:
- Stage IIIA - less than one complete breast is affected
- Stage IIIB - the whole breast is affected, might have spread to issues near the breast, such as the skin or chest wall, including the ribs and muscles in the chest. The cancer may have spread to lymph nodes within the breast or under the arm.
- Stage IV means that the cancer has spread to other organs. These can include the bones, lungs, liver, and/or brain, as well as the lymph nodes in the neck. [16]
Treatment
Chemotherapy: Inflammatory breast cancer is typically treated with chemotherapy.
Radiation therapy: often used after a mastectomy, it uses high-energy beams to kill cancer cells. After treatment, patients may have some side effects such as swelling and redness of the breast.
Hormone therapy: this is used when patients test positive for estrogen and/or progesterone receptors on tumor cells. Hormone therapy is given after surgery and chemotherapy are completed. This is often given for 5 years.
Surgery: surgery is much less useful for IBC than other breast cancers and not supported by strong evidence way. Traditionally surgery for IBC was almost universally avoided, its role is presently beeing reconsidered.[6] A lumpectomy, when only a portion of the breast is removed, is not an option for IBC patients. A lymph node dissection is also recommended over a sentinel lymph node biopsy. Lymphedema, swelling of the arm and the hand on the side of the body where surgery was performed, may be a complication after a lymph node dissection. Reconstruction of the breast may be an option for healthy women after a mastectomy. However, for patients who smoke or have diabetes, complications are more common.[17]
See also
References
- ^ "Inflammatory Breast Cancer: Questions and Answers". National Cancer Institute. Retrieved 2006-12-02.
- ^ "Inflammatory breast cancer". Mayo Foundation for Medical Education and Research. Retrieved 2006-12-02.
- ^ "Facts for Life - Inflammatory Breast Cancer" (PDF). Susan G. Komen for the Cure. Retrieved 2006-12-02.
- ^ Wingo, Phillis. "Population-based statistics for women with inflammatory breast cancer (United States)." Cancer Causes and Control 15 (2004): 321-28
- ^ 11) Gordon, Lisa. "Picture This." CLINICAL JOURNAL OF ONCOLOGY NURSING 5 (2001). EBSCO. Academic Search Complete. 02 Apr. 2009
- ^ a b Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 14580242 , please use {{cite journal}} with
|pmid=14580242instead. - ^ "Inflammatory Breast Cancer Help—Signs and Symptoms." Inflammatory Breast Cancer Association. 02 Apr. 2009 <http://www.ibchelp.org/symptoms/>
- ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 8085857, please use {{cite journal}} with
|pmid=8085857instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 1387777, please use {{cite journal}} with
|pmid=1387777instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 16740744, please use {{cite journal}} with
|pmid=16740744instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 17700572, please use {{cite journal}} with
|pmid=17700572instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 9238713, please use {{cite journal}} with
|pmid=9238713instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 16244790, please use {{cite journal}} with
|pmid=16244790instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 15090727, please use {{cite journal}} with
|pmid=15090727instead. - ^ Attention: This template ({{cite pmid}}) is deprecated. To cite the publication identified by PMID 16198566, please use {{cite journal}} with
|pmid=16198566instead. - ^ "Diagnosis and Staging of Inflammatory Breast Cancer." BreastCancer.org—Breast Cancer Treatment Information and Pictures. 15 Apr. 2009 <http://www.breastcancer.org/symptoms/types/inflammatory/diagnosis_staging.jsp>
- ^ "Inflammatory breast cancer;breast cancer treatment;IBC;breast cancer - nccn.com." National Comprehensive Cancer Network - nccn.com. 13 Apr. 2009 <http://www.nccn.com/inflammatory_breast_cancer.aspx>