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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.

After radiation, am I radioactive? Can I be near my kids?

It is perfectly safe to be around other people including your children after radiation. External beam radiation enters your body, damages DNA in some cells, and some of the radiation exits your body through your back (yup some women have burns on their back). Your cells do not become radioactive. After radiation you can go about your day as you wish/feel able to. Cumulative fatigue from radiation and the rest of your treatment may limit your stamina for some time, especially if you are expecting to go back to a full active life.

Should I get scans after surgery and radiation to make sure they got everything?

It is common to wonder whether the surgeon got all the remaining tumor out. The current standard of care is not to perform scans such as PET or CT scans after surgery and radiation for stage III patients, in the absence of symptoms of metastasis. On the other hand, if you were diagnosed at stage IV (or developed metastatic disease during the course of chemotherapy-surgery-radiation), then it is normal to continue routine scans once you begin systemic therapy again. The exact frequency and type of imaging will be personalized to your disease behavior. Our best advice for overcoming the anxiety from lack of scans is to maintain good communication with your medical oncologist regarding any unusual symptoms or problems you are having.  Your follow-up appointments during the first 2 years should be relatively frequent (every 3-6 months is reasonable for your medical oncologist) and then after that, you might have less frequent appointments if all is well.

If I had a lot of cancer left over at surgery, should I get more chemo afterwards?

This is a very reasonable concern to voice, given that significant amount of residual disease at surgery is a poor prognostic marker.  However, this assumes that more chemo is better than less. Unfortunately this premise is not valid for standard cytotoxic chemotherapy. Cancers that are highly resistant to the cocktails containing multiple types of chemo upfront, are often cross-resistant. In addition, the side effects are cumulative and begin to outweigh the benefits (eg heart damage, immuno-deficiency).  In certain disease settings there are adjuvant clinical trials using newer targeted drugs that are more commonly used in the metastatic setting, earlier in the course of disease for patients with significant residual disease that may be of interest to you in preventing or delaying possible recurrence. Speak to your medical oncologist about whether any of these trials exist that fit your situation.

How often should my port be flushed?

After your chemotherapy and other infusions have been completed your port may be left unused until you decide to get it removed. Most port manufacturers recommend routine heparin flushing at 4 week intervals, however some variation does occur in practice.  A small retrospective study showed that prolonging the frequency to every 3 months did not increase significantly the rate of complications. These potential problems include clots and infections. If the port is not flushed carefully by trained professionals, serious problems may occur. Follow the guidance of your oncologist about when these flushes need to occur.

When should my port be removed?

There is no hard and fast rule. Some patients with anticipated good responses who no longer have a use for their port get it removed at their mastectomy. Others don’t feel safe removing it for a year or more after that to make sure they are still NED (“No evidence of disease”). If you are HER2 positive, then you need it for several months at a minimum after mastectomy while you finish getting the 1 year total of Herceptin.  The port can remain in for months or years more without problems, but it should be flushed regularly if it is not used for infusions or blood draws to reduce the chance of infections and clogging. Some women have some slight discomfort from the port and get it removed as part of ‘moving on’ from cancer.  If you are stage IV, then usually you would keep your port indefinitely since systemic treatment (chemo/targeted therapy) continues after surgery. Speak with your own oncologist who knows your situation to figure out the best removal time for you.

If I had a lot of cancer left over at surgery, should I get more chemo afterwards?

This is a very reasonable concern to voice, given that significant amount of residual disease at surgery is a poor prognostic marker.  However, this assumes that more chemo is better than less. Unfortunately this premise is not valid for standard cytotoxic chemotherapy. Cancers that are highly resistant to the cocktails containing multiple types of chemo upfront, are often cross-resistant. In addition, the side effects are cumulative and begin to outweigh the benefits (eg heart damage, immuno-deficiency).  In certain disease settings there are adjuvant clinical trials using newer targeted drugs that are more commonly used in the metastatic setting, earlier in the course of disease for patients with significant residual disease that may be of interest to you in preventing or delaying possible recurrence. Speak to your medical oncologist about whether any of these trials exist that fit your situation.

What reconstruction options are available to me? How long must I wait?

Breast reconstruction methods fall into 2 categories – autologous reconstruction or implant based methods. Autologous reconstruction methods use your own tissue and include methods such as the DIEP flap or the TRAM flap or a latissimus dorsi. These methods are best for IBC patients.  Implant-based methods involve the placement of a skin expander at mastectomy and gradual stretching of the skin by filling up the expander with fluid.  Once the skin has stretched sufficiently over multiple months, then the expander is removed and replaced by a saline or silicone-filled implant. While this might sound like a more appealing strategy than taking stomach or back tissue, in IBC it is not recommended due to the damage to the skin from the intensive radiation needed. In addition, trying to radiate in the presence of an expander often compromises the radiation plan. This is particularly a problem in IBC patients since radiation is exceptionally important for reducing the chance of a local/regional recurrence.

The next question is timing. Some surgeons like to talk about immediate reconstruction as a way to potentially get the woman back “whole” again more quickly, and avoid the potential mental anguish due to loss of breasts.  But in IBC, whether or not immediate reconstruction should be encouraged for patients remains controversial. Due to the skin involvement and higher rates of early recurrence, reconstruction is recommended to be delayed 1 to 2 years post completion of treatment.  This can be very hard for a woman to hear and one day research might allow earlier reconstruction. For now though, the recommended schedule is waiting at least 1 year, preferably 2 years before doing an autologous reconstruction method that works for your body (DIEP flaps are the most common technique used nowadays, and since many women with IBC have higher BMIs, this can be a benefit as a source of tissue). Speak with a plastic surgeon about what would be best in your situation. Contact us if you would like to join a closed facebook group we run to discuss reconstruction with fellow IBC survivors.   

What are the differences between tamoxifen, and aromatase inhibitors?

Tamoxifen and aromatase inhibitors (AIs) are both examples of endocrine therapy used for ER/PR+ breast cancers to reduce recurrence.  The both act on the estrogen signaling pathway in different ways.  Tamoxifen binds to the estrogen receptor (ER) and stops it from being activated by estrogen.  Aromatase inhibitors such as Femara (Letrozole), Arimidex (Anastrozole) or Aromasin (Exemestane) block the production of estrogen from cells that express aromatase (such as fat cells, uterus, bone, and skin).  The aromatase enzyme converts androgens such as androstendione to estrone, and testosterone to estradiol.

Another difference apart from their different mechanisms is the populations of patients that can take each drug. While tamoxifen works in both pre-menopausal and post-menopausal patients, AIs are usually used in post-menopausal patients since once the ovaries stop working, then the main source of estrogen in the body is from peripheral fat stores. This process of androgen conversion to estrogens in the fat cells can therefore can be inhibited with an AI. In pre-menopausal women, the ovaries are the most active source of estrogen; hence if the ovaries are still functioning, AIs are useless in affecting overall estrogen production and signaling. Therefore, if a pre-menopausal patient cannot take tamoxifen or wants to take an AI for other reasons, they must have chemical ovarian suppression or have their ovaries removed surgically for this approach to work. Ovarian suppression is accomplished via a monthly injection of Zoladex or Lupron.


To where can IBC metastasize?

IBC can spread to virtually anywhere in the body, but there are some locations that may be more likely. Bone is a common location for metastasis, and in IBC bone metastasis acts more aggressively than other breast cancer bone metastases. Other common sites are lung, liver, skin and distant lymph nodes. If you have health concerns regarding other anatomical locations that are persistent and not explained by your known health issues, IBC recurrence should be ruled out.

What are the signs of IBC recurrence that I should be aware of?

Some symptoms of metastatic IBC may include:

  • bone pain that does not go away
  • shortness of breath, chest pain, or cough
  • pain or discomfort under the side of the ribcage that won’t go away
  • abdominal pain or bloating
  • lack of appetite
  • unexplained weight loss
  • neurological pain or weakness and headaches
  • confusion
  • irregular gait
  • skin rash
  • vision issues

These symptoms may be possible signs of metastasis to the bone, lung, liver, brain, skin or other parts of the body. However, this does not mean that every woman who experiences them has metastatic cancer. There can be a variety of other reasons why you might experience the above symptoms that may have nothing to do with cancer of any kind. Short-lived aches and pains and lumps often have nothing to do with cancer, but can be a normal part of aging or even an injury. Cancer survivors are not immune to other mild illnesses too. If issues last more than two weeks, please contact your doctor to determine the cause of the symptoms.

If I have a bilateral mastectomy, how can IBC come back?

Even in the absence of breasts or reconstructed breasts, IBC can return in distant sites. This is because tumor cells from the primary tumor escaped and survived chemotherapy. They often remain dormant in distant sites for months to years, and we don’t really understand what triggers them to start growing again to cause detectable metastases.

If IBC spreads, is it as aggressive/fast as it was in the breast?

It is hard to provide a generic answer as every cancer is unique and constantly evolving. We do know there is a spectrum of IBC from slow-growing to fast growing. What we generally see is that a fast-growing primary tumor tends to act equally aggressively when it comes back. For slow-growing indolent cancers (many triple positive tumors behave this way), metastases can be slow in the beginning, but over time depending on what mutations arise, the nature of the disease can become less treatment-sensitive.

Should I get my ovaries removed to reduce recurrence?

This is an area of frequent confusion among breast cancer patients. There are 3 potential reasons we should discuss regarding ovarian removal. For ER+ IBC survivors, removing the ovaries can be a way to reduce circulating estrogen levels. Free estrogen could promote survival/growth of micrometastatic clones if endocrine therapy is not being taken correctly. Another potential benefit from ovarian removal in young IBC survivors would be the ability to take aromatase inhibitors. In randomized studies AIs have been shown to reduce recurrence more than tamoxifen in the post-menopausal setting – so those at highest risk for recurrence may want to consider this strategy after discussing with their medical oncologist the potentially more severe side effects from sudden menopause. Ovarian removal is not the only option though, so discuss with your medical oncologist in detail about the pros and cons of this decision.

The ovaries themselves are infrequent sites for metastases, so removing them does not significantly impact potential death from metastatic breast cancer. Intriguingly, lobular breast cancers are more like to metastasize to gynecological organs like the ovaries than other breast cancer histologies such as ductal cancers.

The other important reason why ovaries might be recommended for removal is if you are at a high risk for primary ovarian cancer (different from metastatic breast cancer in the ovaries). This is because many women who are considered high-risk for breast cancer due to BRCA1/2 inherited mutations also have a much higher-than-average risk of developing ovarian cancer (20-40% lifetime risk, compared with 1.5% in the general population). It is important to know however that BRCA mutations are only found in 1:300-1:500 of the general population and out of all breast cancers, less than 10% are due to these mutations. Other genes, both known and unknown, as well as environmental/behavioral factors are responsible for the majority of IBCs.  Regardless, coming back to this question, if you do not already know your BRCA1/2 mutation status, and you are concerned about your ovarian cancer risk, talk to your doctor about seeing a genetic counselor (a health professional specially trained to take your family history of different cancers, and provide information and advice about inherited conditions). If after meeting with a genetic counselor you are found to be at high risk of having an inherited mutation, you may decide to perform genetic testing. If you decide to get tested for a germline BRCA1/2 mutation then ovarian removal may be one of the methods to reduce your risk of developing ovarian cancer later (and therefore having to go through chemotherapy and surgery all over again!)

I need dental work, but I am on bone-protecting medications (Denosumab/bisphosphonates). What do I need to know?

Both bisphosphonates and Denosumab have a risk of inducing jaw osteonecrosis if invasive dental work is done while a patient is taking these drugs. This risk is less than 2% but if this happens, it may be a significant cause of pain and suffering that is difficult to treat. Therefore prevention of this problem is recommended.  Invasive dental procedures (including tooth extractions or placement of dental implants) should be avoided if at all possible. However, other less invasive procedures can be completed such as dental cleaning, repair of cavities, crown placement, or root canals. If a dental extraction or other invasive procedure is absolutely essential during therapy, the drug should be held for at least 8 to 12 weeks if possible, and restarted only after complete mucosal healing has been observed. It is absolutely critical that the dentist is informed about a patient’s treatment with these drugs and the timing so that they can advise appropriately

What foods should I eat so I can stay cancer-free?

As much as we would like to have a recommended diet to prevent recurrence, unfortunately there is no guarantee. Maintaining a healthy body weight and eating a nutritionally-balanced diet are indeed recommended as ways to increase the likelihood of remaining disease-free, but no magic food items exist to stave off cancer. Biology is king… You might read about certain types of diet to alkalize your body for example – however these are not recommended and some may actually harm you in the long term.  If you are concerned about your diet, perhaps your oncologist can refer you to a nutritionist if you are being treated at a large cancer center. Further resources will be added to the app over time as well.

 What is the difference between hospice and palliative care?

Palliative care is care aimed at making the patient more comfortable for example dealing with side effects of cancer and its treatments. Palliative care specialists can be engaged at any time after diagnosis – and can be consulted even during curative therapy.  Hospice care is end-of-life care, when a patient and their oncologist decide there are no more treatments available with a greater chance of helping than harming them.  There are elements of palliative care that is incorporated in hospice care, but hospice care is entirely different in nature from palliative care that can be provided on an outpatient basis as needed throughout treatment and survivorship.

Long-Term Side Effects & Concerns

What is lymphedema and what can I do about it?

Lymphedema is a collection of fluid that causes swelling in extremities and even the upper body. Breast cancer survivors who have had an axillary dissection or axillary radiation often have upper arm swelling due to the removal of the lymph nodes which would normally participate in lymph drainage.  Lymph nodes play important roles in filtering the lymph fluid to trap foreign substances so they can be removed by white blood cells. The fluid can also build up in the upper body – a form of lymphedema known as truncal lymphedema.  The symptoms of lymphedema include feeling as though your clothes or jewelry are tighter than usual; a feeling of fullness in your arm and less flexibility in your joints.  If you think you have lymphedema, there are various types of compression garments such as sleeves. For more serious cases of lymphedema, you may need to learn to wrap your arm from a lymphedema specialist or use a pump.  Some women find manual massage of the affected arm to be helpful. Even if you do not already have lymphedema it is wise to have a sleeve for use when you travel by plane, since the changes in air pressure can trigger swelling or pain.

Hormonal therapies have made my vagina dry and painful. I am scared to use estrogens that might cause recurrence. What types of creams/moisturizers are safe for me to use?

Unfortunately vaginal dryness is a common problem in breast cancer survivors – 30-60% of women have this problem at some point during endocrine therapy. There are several non-hormonal choices for moisturizers and lubricants that should be tried before estrogen-containing products. Replens is one common product that is available over the counter for vaginal dryness that does not contain hormones.  Other examples of products that others have found helpful include Hyalu GYN gel (hyaluronic-acid based), vitamin E suppositories, and a variety of natural oils such as coconut oil or almond oil.  

On the topic of vaginal estrogen creams such as Estrace, the systemic absorption is very low (but detectable in patients taking aromatase inhibitors using very sensitive assays). Whether this amount is enough to increase recurrence risk is questionable – the one prospective study done in >1400 patients found no increase in recurrence after 1 year of use. In patients who are taking tamoxifen, this extra estrogen is unable to influence cell growth pathways normally regulated by ER because ER is blocked by tamoxifen. Some oncologists do not feel any extra estrogen is safe in ER+ breast cancer survivors, but others are more pragmatic about this, especially if other methods have not improved the problem which may be impacting overall quality of life. In summary, the use of estrogen creams is a very personal decision taking into account both recurrence risk and the severity of the vaginal problems.

At what point after IBC is it safe to get pregnant if I desire a child/more children?

This question is reasonable from 2 perspectives – the safety for a potential fetus as well as determining whether pregnancy adversely affects recurrence risk.  Overall the limited data we have on young women survivors of breast cancer who subsequently become pregnant, suggests that these women have equivalent outcomes with respect to recurrence than others, matched stage for stage.  In addition, babies of breast cancer survivors do not suffer harms from the chemotherapy that we can tell.  However, not all women remain fertile after chemotherapy especially those approaching the age of natural child-bearing becomes less likely.  In terms of advice for IBC patients, certainly while you are on active treatment (whether that is chemotherapy or Herceptin) it would be unwise to become pregnant. After treatment, your childbearing decisions must take into account your risk of recurrence and your hormone receptor status in the tumor.  If you are ER or PR positive, you will be taking endocrine therapy for a minimum of 5 years – and even though it is physiologically possible to be fertile while taking tamoxifen, it is important not to become pregnant while taking it – completing the course of endocrine therapy is important for reducing recurrence. Also be aware you may begin menstruating, but this may or may not correlate with being able to have a child. If you are on an aromatase inhibitor with ovarian suppression (and taking the injections on schedule), the risk of pregnancy should be very low.

My treatment is finished, but I am not back to normal. Is this usual? How long does it take to feel normal again?

Congratulations for completing treatment!  So you rang that bell but life is not as you expected?    It is important to realize your body had been put through a great deal, physically and emotionally.  Some time will be needed to recover and process as you get back to your old self.   You might have experienced weight gain, fatigue, chemo brain, or any number of side effects that might be with you for awhile.  Some call this a “new normal” but it might not feel so normal to you.  Give yourself some time but also address any issues you are feeling.  Check with your doctors to see if any medications could be causing side effects, or medication is needed to control side effects.  Also post cancer treatment, could be a good time to revisit your diet and exercise patterns to see if food or exercise changes can give you more energy and help increase stamina  to help your future can be as happy, healthy and comfortable as possible.

Miscellaneous Questions

What is a liquid biopsy? How are they used in IBC treatment?

A liquid biopsy is a generic term for 2 types of blood tests that may be used for monitoring the course of cancer/recurrences. One of the tests looks for circulating tumor cells (CTCs) which have broken away from the primary tumor and are in the blood. These tumor-derived cells may or may not lead to a subsequent metastasis since there are multiple steps involved in establishing a new growing tumor in a different environment than the breast. What we have learned from retrospective studies is that  even 1 CTC may lead to a metastasis, and is therefore a negative prognostic factor.  Research is ongoing to design new methods of obtaining biological information from these cells – for example sequencing the DNA from them or examining HER2 status.  Currently (in fall 2015), CTC analysis for clinical use is mainly enumeration (counting) them. In the future it is possible that the number of CTCs may be used to track the success of therapies in the metastatic setting.  

The second type of test involved in a liquid biopsy is a test for circulating DNA that derives from tumor cells. This DNA can be sequenced to discover mutations that may be therapeutic targets for future treatments.  Since tumors are not clonal (ie different cells can contain different mutations), the amount of each mutation can be tracked over time, which can tell you whether the targeted therapy is working and give you an overall picture of the molecular biology of all the different metastases, not just the ones that are in accessible locations amenable to biopsy. In addition, over a period of time, as tumors evolve new mutations to survive therapy, these new mutations can be discovered and give oncologists insight into how tumor cells resist drugs. New drugs are being developed that target mutant proteins, so it is possible you may find clinical trials in the future to target these ever-changing survival pathways.

What are tumor markers and should my oncologist be using them to track me?

Tumor markers are substances that can be detected in the blood that may correlate with the presence of cancer – often months before metastases are radiologically visible.  In IBC and other breast cancers, there are 3 different tumor markers that may be used – CA15-3, CA27.29 and CEA.  CA15-3 and CA27.29 are glycoproteins that are related to each other (both derived from the MUC1 gene). It is important to realize not all tumors express these markers, and for those patients with negative tumors, measuring these markers is not meaningful. For others, the marker is a very good indicator of overall disease burden.  Elevated tumor markers may occasionally be spurious. Up to 20 percent of patients successfully treated with systemic therapy may experience a transient increase (marker “flare”) during the first 1-2 months after beginning a new treatment, presumably due to release of antigen from dying cells. Patients with abnormal liver function may also have falsely elevated marker levels because they are cleared by the liver. Other explanations also exist for elevated markers. Therefore it is important to speak with your own oncologist about your results to make decisions together about your situation. Usually a single elevated value is not enough by itself to instigate a change of therapy – rather a trend of rising values above the normal level may be a signal to perform imaging tests. In addition, physical exam and history taking at each appointment is critical for determining whether your disease is progressing/recurring.

What is the difference between genetic and genomic testing?

Genetic testing is used to look for genetic predisposition genes that might have contributed to your cancer developing. It is done by sequencing normal tissue such as white blood cells. Commonly genetic testing is done to look for BRCA1 or BRCA2 mutations if you have a significant family history of breast, ovarian and certain other cancers. There are multiple other genes with less strong risk that are now being examined alongside BRCA1/2.  

Genomic testing is different entirely from genetic testing since the focus of genomic testing is on the tumor itself. Genomic testing nowadays usually consists of providing a biopsy for next-generation sequencing of a panel of genes (anywhere from a small targeted panel of 50 cancer-related genes, to 300-400+ genes, some that have no targeted drugs available). The goal of genomic testing is to find actionable mutations that could suggest new therapies that might work for your cancer. Genomic testing can be done either by commercial companies (such as Foundation Medicine) or by major academic institutions such as the University of Texas MD Anderson Cancer Center, Dana-Farber Cancer Institute or Memorial Sloan Kettering Cancer Center. Speak with your oncologist about the best option for you.

Can I get vaccinations such as a flu shot while on chemo/in treatment?

Yes, most likely it is safe for your to receive vaccinations during chemo – but check with your oncologist who knows your unique situation.  There is some theoretical concern that because chemotherapy decreases your white blood cells, that there may be less of an effect of a vaccine.  However, even moderate protection can benefit you and your family, and indeed studies that have looked at the robustness of immune response have suggested that many cancer patients can mount almost equal responses to vaccinations. Cancer patients are at an elevated risk of flu and may have higher mortality rates from influenza infection than the overall population. In addition, getting sick may delay your needed cancer treatments.  Taking a trivalent inactivated influenza vaccine therefore is of overall benefit – but live attenuated vaccines for flu (less common) are not recommended while you are in treatment. The ideal timing for vaccination would be at the furthest point from treatment during a given cycle, assuming your WBC count is above 1000 cells/mm2.