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General IBC Questions

How many cases of IBC are there in the US?

Unfortunately it is difficult to know the exact number because of a few problems. One is that IBC does not have a medical encoding number (ICD code). Another issue is misdiagnosis in both directions – IBC is missed, and sometimes IBC is diagnosed but is really a locally advanced breast cancer.  Despite these issues, it has been estimated that IBC accounts for 1-5% of all breast cancers in the US each year. Given an estimated 230,000 new cases of breast cancer in 2015, this means it is likely that 4600 or more of these are IBC.

If IBC is just in the skin why is it more aggressive?

IBC is not just in the skin. It may be scattered throughout the breast parenchyma (lobules/ducts). The migratory/invasive nature of IBC allows it to invade the skin and produce the visual symptoms of IBC. The reasons why IBC is more aggressive than non-IBC are not well understood. Molecular studies on just tumor cells has failed to identify reproducible gene expression differences, leading to the hypothesis that IBC may be more aggressive because of the role of the tumor microenvironment (ie host factors). These are being actively studied, so that in the future these factors may be targeted or used as early diagnostic markers.

Are there any genetic markers for IBC? (i.e. inherited genes that lead to IBC)

There are no known specific genetic predisposition genes that predict IBC. However, genes that are known to increase risk of breast cancer may also increase risk of IBC (eg BRCA1, BRCA2, PALB2, p53, LKB1 etc). Unfortunately there is no data on the low-frequency mutations that may be linked to a family history of IBC. No studies have been done on the rare families that have more than 1 IBC case, despite this potentially being a valuable way to identify novel familial risk factors for IBC. A retrospective study (presented as a meeting abstract) performed at MD Anderson, using patients that were referred for genetic counseling based on a family history of breast and/or ovarian cancer, found that the genes BRCA1 and BRCA2 are mutated at a similar frequency in this cohort of IBC patients (35.9% vs 26.1% for non-IBC – not statistically different). Note that the true incidence of mutations is much lower since we do not currently test all patients for these mutations. Thus it may reasonable to extrapolate that the current overall breast cancer population risk of BRCA1/2 mutation applies to IBC as well. This means that only 5-10% are likely due to hereditary factors that are known today. More than half of IBC patients do have a family history of breast cancer so more genes may be identified in the future – these histories may also elucidate shared environmental factors that predispose to IBC development. Cancer is complex – the vast majority is not driven solely by a strong high-risk gene such as BRCA1.


What makes the breast ‘red’ with IBC?

The red appearance of the breast with IBC is due to the tumor cells which block the lymphatic vessels surrounding the breast. The breast may start off as pink and become progressively redder or even purple as the tumor progresses.

Why are mammograms notoriously ineffective in detecting IBC?

There may be a few reasons for this. One is that IBC doesn’t form a discrete lump that can be seen easily and measured. However, IBC does have other features that can be observed on a mammogram if the radiologists are trained on what to look for. These signals of IBC include trabecular distortion, skin thickening and retraction of the skin and nipple. Another reason why a regular screening mammogram might not pick up IBC is that there is a relatively short latency between IBC initiation and full-blown disease – generally within 1-3 months of first symptom the entire breast may be noticeably involved. IBC diagnosis mid-screening cycle is quite common. Another reason is that in younger women especially who have denser breasts, mammograms lose sensitivity because more of the breast is white on a mammogram.

Can IBC be confirmed on a biopsy alone?

No, a pathologist who just looks at a slide from an IBC patient cannot tell it is definitely IBC. This is because IBC is a clinical diagnosis. A biopsy would only confirm the presence of an invasive breast cancer. Your pathology report will state the histological subtype (such as invasive ductal carcinoma or invasive lobular carcinoma). If the pathologist has looked at the clinical record, he/she might note likely inflammatory breast cancer, but do not be alarmed if inflammatory breast cancer is not on your pathology report.  The tumor cells themselves do not look any different from other high grade breast cancers.  In IBC one difference is the higher prevalence of lymphovascular invasion and nests of tumor cells in the skin (called dermal tumor emboli). Your report may or may not mention those aspects.

What is the difference between grade and stage?

Tumor grade is the description of a tumor based on how abnormal the tumor cells look under a microscope. It is an indicator of how quickly a tumor is likely to grow and spread. If the cells of the tumor and the organization of the tumor’s tissue are close to those of normal cells and tissue, the tumor is called “well-differentiated.” These tumors tend to grow and spread at a slower rate than tumors that are “undifferentiated” or “poorly differentiated,” which have abnormal-looking cells and may lack normal tissue structures. Based on these and other differences in microscopic appearance, doctors assign a numerical “grade” to most cancers. Breast cancers can be grades 1-3, with grade 3 being the most aggressive. Most IBC is grade 2 or 3.  

Tumor grade is not the same as the stage of a cancer. Cancer stage refers to the size and/or extent (reach) of the original (primary) tumor and whether or not cancer cells have spread in the body. Cancer stage is based on factors such as the location of the primary tumor, tumor size, regional lymph node involvement (the spread of cancer to nearby lymph nodes), and the number of tumors present. IBC is always either stage IIIB, IIIC or IV.

What is the significance of ER, PR and HER2 status for my treatment?

ER and PR status are important in deciding upon whether endocrine therapy is needed after chemotherapy, surgery and radiation.  If even 1% of tumor cells express ER or PR (also known as HR positive/HR+ tumors), some type of endocrine therapy would be recommended to decrease risk of recurrence.  HER2 is particularly important to know before beginning treatment since the chemotherapy protocol is designed with this in mind. HER2 positive tumors receive HER2 targeted therapy along with chemotherapy. The current standard in the US for neoadjuvant treatment for HER2+ tumors includes Herceptin, Perjeta (Pertuzumab) and a taxane. There are a few options for the other drugs used. HER2 negative tumors do not receive Herceptin or Perjeta since the target of these drugs are not present at high levels.  In terms of chemotherapy choices for HR+ or TN (triple negative) tumors, a number of cocktails may be used and these are usually selected based on the treating medical oncologist’s preference.

What is the difference between stage IIIB, IIIC and IV IBC? What difference does it make in my overall treatment plan?

Breast cancer staging (including IBC) is performed both to guide treatment and inform the patient about their prognosis. Staging incorporates both tumor size as well as presence of tumor cells in regional lymph nodes and presence/absence of distant metastasis. If the tumor has remained localized to the breast and regional lymph nodes then your stage is either IIIB or IIIC. If you have tumors elsewhere in the body, then you are stage IV.  To distinguish between stage IIIB and IIIC, your oncologist will use the results of your PET and/or CT scans. Generally IIIC means you have further lymph node involvement such as in the superclavicular nodes in the neck.

How should I tell friends and family about my diagnosis? What should I tell my kids?

This is such a personal question and the only answer is what is best for you, the person living with this disease.  Does this mean your diagnoses doesn’t impact others? Of course not.  Family, friends and even children, could be viewed as “co-survivors” and feel as if they are in treatment with you.  If you are comfortable sharing details, please share.  Then your loved ones can know your concerns and needs and be of best support.  Have a “hoverer” in your life?   Someone who helps too much and you feel a loss of control?  Lay down some guidelines and boundaries – see this article for suggestions on doing so.  If you have the opposite situation i.e. someone important in your life who seems disconnected, counseling might be needed to help allow honest and helpful dialogue.  How to discuss cancer with children varies greatly by age and level of maturation.  Just because a children might be older and shows a great deal of maturity, does not mean they are ready to have a cancer conversation on an adult level.  Time spent with a child will allow questions to come up naturally in conversation and questions can be addressed in real time.  Children will sometimes let their imagination get the best of them or sometimes blame themselves when an adult is ill.  Honest, direct but age appropriate discussions can be of great value to all parties.  Also consider some of the wonderful programs for family and children via your hospital or even summer camp programs for children, like Camp Kesem.

Is IBC a death sentence?  

Absolutely not!  IBC is a very serious disease however there are women on record who have lived 20+ years post an IBC diagnoses.  “Hope always” is our motto. Hopefully the information in this app can help you receive the best treatment possible to maximize your chance of long-term freedom from IBC.


What is tri-modal treatment and why is it important in IBC?

Tri-modality treatment in IBC means that treatment consists of 3 modalities – chemotherapy, surgery and radiation.  Chemotherapy is important because it targets both the tumor cells in the breast and skin, as well as tumor cells that have escaped. Chemotherapy is necessary to make the breast suitable for surgery – i.e. reduce the skin involvement so that the surgeon does not need to cut directly into diseased skin. Surgery is important to remove the bulk of the breast tissue and skin which usually contain some disease.  Knowing how much disease (if any) is left over is a prognostic factor. Radiation is the last piece of treatment, and is an important mop-up strategy for scattered left-over tumor nests.  Chemotherapy is an example of a systemic therapy, while surgery and radiation are local therapies. Both of these types of therapies have their roles in IBC care.

Why is chemotherapy given first in IBC treatment?

IBC is an example of a locally advanced, non-operable breast cancer. This means that cutting into the breast to perform a mastectomy is not advisable due to the presence of widespread tumor emboli.  The purpose of chemotherapy is to shrink these tumor nests as much as possible. Also IBC is known to spread early in the course of disease systemically. Chemotherapy also targets these tumor cells that have migrated away from the primary tumor; these may be micrometastatic (not visible on standard imaging).  This is another reason for prompt initiation of chemotherapy rather than upfront surgery.

What are targeted therapies?

Unlike cytotoxic chemotherapy which kills all dividing cells indiscriminately, targeted therapies are drugs that are specifically designed to inhibit the function of a target protein. Ideally the target is more specific for cancer cells than normal cells, or at least expressed at a much higher level. Some examples of such drugs used in IBC include HER2 targeting antibodies or small molecule inhibitors (Herceptin, Perjeta, Lapatinib/Tykerb, Neratinib etc) or Everolimus (Afinitor) that targets mTOR. Hundreds of targeted drugs are in clinical trials for cancer.

What is a port and why would I want one?

A port is a small disc made of plastic or metal about the size of a quarter that sits just under the skin. A soft thin tube called a catheter connects the port to a large vein. Chemotherapy and other liquids are given through a special needle that fits into the port. You also can have blood drawn through the port possibly saving some wear and tear on veins in your arms as you get treatment.  One good tip to know of you are person of petite size, ports do come in more that one size, so discuss this with your surgeon.  

Can I do chemo without a port?

Yes it is possible to have your chemotherapy given to you via a peripheral IV. While this might help you begin chemo more promptly, over time, your veins may become damaged and more difficult to find. In addition, some of your chemotherapy drugs can cause tissue damage if the needle accidentally leaks the drug into your skin. A port prevents these from being a problem, and also makes it easier to do routine blood draws.

If I have a complete response to chemotherapy by imaging, do I need to have a mastectomy?

Yes surgery is recommended for all stage III IBC patients who have at least a partial response to chemotherapy. If you have a very good response to chemotherapy, this is excellent news for your long term prognosis. The limitation with relying upon imaging for decision-making is that current imaging modalities are not perfect. Even PET scans which may be the most sensitive type of scan, still require a few millimeters of tumor to detect anything. In addition, due to the scattered/webby nature of IBC, it is impossible to know that all of the tumor is dead without removing the breast surgically and examining it under the microscope for the presence of live tumor cells. If disease is left in the breast, it will just begin growing again once chemo has been stopped, and then potentially seed distant metastasis which will eventually kill you. Ultimately it is much safer to remove the breast which is an obvious source of potential metastases, and then follow through with the post-mastectomy radiation that is part of the consensus IBC treatment protocol.

Why can I not have a sentinel node biopsy at surgery like other breast cancer patients?

Sentinel lymph node biopsy (SLNB) is a newer less invasive way surgeons have developed to stage the regional lymph nodes in different cancers including breast cancer based on the principle that if a breast tumor is injected with a blue radioactive dye, the dye would naturally drain to the first set of lymph nodes that would be the most likely sites for metastatic spread. The concept is that if the blue dye containing nodes (which are evaluated during the surgery by the pathologist) don’t contain tumor cells, the likelihood is that the other nodes are also negative. Based on large studies showing their accuracy and safety in early breast cancer, SLNBs have been accepted as the standard of care to evaluate axillary lymph node status in patients with early breast cancer, as a way to reduce the morbidity from full axillary dissections in patients with limited/no lymph node involvement. However SLNB is not recommended for patients with IBC because of lymphatic blockage by tumor cells and the unreliability of the SLNB procedure after neoadjuvant therapy in the IBC setting. In one study, eight patients with IBC underwent SLNB after neoadjuvant chemotherapy. The rate of identification of SLNs was 70% and the false-negative rate was 40%

[67]. This unacceptably high false-negative rate demonstrates the unreliability of SLNB in IBC. In addition, because of the lymphatic issues, sentinel node biopsies frequently fail in IBC patients due to non drainage of the blue dye, making a full axillary dissection necessary anyway.

Do they have to remove all my lymph nodes?

Axillary lymph node dissections are standard of care in all lymph node positive breast cancer patients, including IBC. Since the sentinel lymph node biopsy method is suboptimal in IBC, even if you are in the minority of clinically lymph node negative patients at diagnosis, the entire axilla must be examined for complete staging information. The purpose of determining how many lymph nodes are still pathologically positive for tumor cells is primarily to provide prognostic information, but also helps by removing sources of future metastases/local recurrences. So yes. all of your axillary lymph nodes that reside in what are referred to as level 1 and level 2 must be removed. Level 1 and 2 means all the nodes that lie below/in front of the pectoralis minor muscle. Level 3 nodes which are above the pectoralis minor muscle used to be removed, but they are no longer included because this increases the lymphedema risk considerably without providing any additional prognostic value.

How soon after chemotherapy should I have surgery?

The waiting period between chemotherapy and surgery is about 4 weeks.  This allows time for your immune system to recover from the chemotherapy.  If your surgeon is rushing you to surgery much before this time, with the concern that the IBC may begin to spread again, consider carefully whether this is the optimal plan. If your response to chemotherapy is poor and the surgeon must cut through actively diseased skin, the likelihood for a long-term disease-free life is very small. A second opinion with an IBC specialist center may help guide the decision of whether to go ahead with surgery or receive more, potentially different systemic chemotherapy or radiation.

What is the difference between Herceptin and Perjeta? Why are both needed?

Herceptin is an antibody against HER2. Perjeta is an antibody that blocks HER2 from binding to one of its co-partners in crime, HER3.  Together these 2 antibodies inhibit HER2 signaling better than Herceptin alone, and blocks HER3 from being active. HER3 activity can be a resistance mechanism to Herceptin.

What should I take to my mastectomy? How long will I be in the hospital?

A mastectomy without immediate reconstruction (i.e. the recommended surgery for IBC patients) is usually a short day/overnight surgery – 2-4 hours from start to being returned to recovery/observation room. If all goes well, patients usually get to go home the next day. So a long list of items to bring is unnecessary. One important thing to note is that both pajamas and a casual shirt with buttons or a zip on the front should be brought, since raising arms will not be possible shortly after axillary surgery.  A small pillow or 2 may be helpful for the ride home.

How long do my drains need to stay in?  

This varies from patient to patient.  Surgeons will often say 7-10 days but 2 to 3 weeks is not uncommon. The timing for drain removal is based on the output of fluid but also with caution to not leave them them in so long to allow an infection to set in.  Maintain your fluid log as instructed by your surgeon to insure the drains are removed at an appropriate time based on your body. Importantly, the next step in IBC treatment which is usually radiation cannot begin until the drains can be removed.

How do I interpret my pathology report?

You will receive at least 2 different pathology reports during your cancer experience – one at diagnosis which will describe the biopsies and a full final report after surgery on the removed breast(s) and lymph nodes. Breast cancer pathology reports are one of the more complex pathology reports and can seem quite overwhelming at first glance. Ask your doctor or nurse to explain any terms you are unfamiliar with, and what that means for your treatment or prognosis.  Both types of reports will tell you what histological type of breast cancer you have. Breast cancers are almost all epithelial cancers that arise from glandular tissue. Breasts have lobules, which make the milk and ducts which carry the milk to the nipples. Depending on which type of cells the cancer most resembles, you get a designation of “ductal carcinoma” or “lobular carcinoma” or mixed. There are also a few very rare subtypes that IBC can resemble.  About 85% of IBCs are ductal carcinomas.  The subtype does not affect your treatment, but knowing your subtype gives some additional information about potential biological differences. Lobular carcinomas have some unusual metastatic spreading patterns, such as to the GI organs and gynecological system, but overall the rate of metastasis is not different between ductal and lobular cancers matched stage for stage.

When you are diagnosed, you will be most interested in knowing the aggressiveness of the disease and your ER, PR and HER2 status. Aggressiveness is semi-quantified by a grade, usually 2 or 3 (with 3 being high grade) as well as comments on the punch biopsy sample mentioning tumor cells that have invaded local lymphatic vessels or blood vessels (known as “lymphovascular invasion”). If you see the word “pleomorphic” that means different – ie pleomorphic nuclei means that the tumor cells have nuclei that look different. This is a feature of a high grade cancer.  In the US, ER and PR are usually reported on 1-100 scale representing the percentage of positive cells for these proteins. Any positive number is taken as a sign that your cancer may be driven by the hormones estrogen and progesterone, and therefore endocrine therapies may benefit you.  HER2 testing may be done via 1 of 2 methods. If the test was immunohistochemistry (IHC) then the results are reported as 0, 1+, 2+ or 3+. If you are 3+, then you will be classified as “HER2 positive”. The 2+ status is a grey area, and the guidelines state that the other test should be used to confirm whether HER2 gene copy number is increased.

The mastectomy pathology report is more complex. There will be a lot more details about what was found in each piece of the tumor – since it must be chopped up into small pieces to be processed and made into slides. Not only will you have breast tissue among these specimens, you will have lymph nodes and surrounding tissue. The summary should include the same factors as the diagnosis report as well as the number of positive lymph nodes out of the total number that were removed. If a lymph node metastasis has burst out of the lymph node, then this is called extra-capsular extension, and should be noted. This is a sign of additional aggressiveness and lack of response to chemotherapy. Another thing to note about the breast is that when it is removed from your body, it is marked by different colors of ink representing the orientation. The ink serves as a way for the surgeon to know where to look if the pathologist says that the edge is not free from cancer. The edge is called a margin. If there is cancer in the inked area, then the margin is “positive” and this is not good! The surgeon will be asked to cut more out if this is the case. Your report should state that the final margin is free of cancer. If the margin cannot be made “clean” – this is an important fact for you to glean from your report, since this should influence your radiation and your potential recurrence risk.

The one section of the report that you may be less interested in is what the tissue looked like by the naked eye. This is called the gross description. Microscopic cancer cells can’t be seen by eye unfortunately.

My ER/HER2 status changed after surgery? Why? Is it real? What does this mean for my treatment?

A change in marker status between your biopsy and final pathology after surgery is not uncommon. You may wonder why, and in most cases we do not know for definite. However what we do know is that a biopsy is only a small sample that may only contain a few thousand tumor cells. This is particularly true in IBC because there may not be a solid mass to sample, and the tumor cells may be in small nests throughout the skin.  Another very important thing to know is that not all tumor cells are alike, and so one area may be HER2 positive while the rest is HER2 negative.  If you sampled each area, you could get a different answer.  Therefore at final pathology a larger area of tumor will be examined, and therefore could pick up differences like this.  The scientific terminology for this is “intratumor heterogeneity”.  Another difference may be the relative resistance or sensitivity of different clones to therapy – the surgical sample will only tell us about the cells that survived chemotherapy and targeted therapies (if relevant). We know in general that some ER+ cells are more chemo-resistant (and hormone-sensitive), and so a greater portion of these may be leftover than at the beginning.  So in summary yes, the differences are indeed real.  As for the meaning of these changes, usually if you gain a marker, then you will receive extra drugs that target these proteins (ie endocrine therapy or Herceptin). This is because the potential harms from not treating exceed the tiny risk of unnecessary treatment. In the case of becoming TNBC, the situation is a little more complex. Most oncologists would continue the treatments that have begun or were recommended based on biopsy, in case the already disseminated micro-metastatic cells still have these markers.  However, there is no randomized high quality data that this is the best approach that improves survival.  As with many decisions, your oncologist should personalize the approach based on your circumstances.

Should I get an Oncotype test? What does this test tell you?

You may have heard or read about the Oncotype DX test that is sometimes in used in early stage breast cancer. It is not relevant in IBC. The Oncotype DX test is used in patients with early stage cancers that are removed surgically shortly after diagnosis. The reason this test was designed was to identify those patients who do not require systemic chemotherapy because their risk of recurrence is too low to derive a significant benefit. Oncotype DX is a molecular gene expression test that quantifies the aggressiveness of the breast cancer and provides the oncologist a score that can be related to a risk of recurrence. If the score is low enough, then chemotherapy can be skipped, and since these patients are usually ER+, endocrine therapy is the treatment recommended after surgery.  Since in IBC, everyone gets chemotherapy upfront, there is no use for this test.

How soon after surgery should I begin radiation?

About 4 weeks is about the average recovery period between surgery and radiation.  Sometimes range of motion can be impacted by the modified radial mastectomy and if your drains are in for a longer period than usual, sometimes radiation must be postponed.   Your physician will suggest exercises to help retain or regain range of motion, so you can lay comfortably with your arm elevated or extended while receiving radiation.

 I’ve read about some IBC patients getting twice a day radiation? Is this standard or how do they decide?

Radiation is a very important aspect of IBC local treatment. For years, the sub-group of IBC patients who made it to radiation, received the same schedule and dosing of post-mastectomy radiation as regular non-IBC patients. However this strategy did not always provide optimal disease control and resulted in both poor survival and morbidity due to skin metastases. Unfortunately IBC cells are intrinsically more radiation-resistant than other breast cancer cells  For this reason, MD Anderson’s radiation oncologists decided to escalate the total dose received by 10% on average, and reduce the recovery time between treatments so that IBC cells could not start growing again once the DNA damage by radiation is repaired.  This is what led to twice a day treatments.  Upon further research, we have learned which patients really benefit from this more intensive treatment – that is patients that are younger (less than age 45), patients who had tumors that responded poorly to chemotherapy or patients who had surgery and had either positive or close margins (sometimes not possible to avoid this). If you lack all of these high-risk features, then once a day radiation treatments up to a total dose of 66Gy is sufficient. The number of treatments varies a bit from place to place, but generally the idea is that you get a total of 50Gy to the entire chest wall and then a boost of 10-16Gy to the scar line. The scar line is targeted because it is the most susceptible area for local recurrence. You will have radiation every weekday until it is finished. Occasionally you might have to take a break towards the end if your skin breaks down too much.