A New Rhabdo Diagnosis
A New Rhabdomyosarcoma Diagnosis?
If you or a loved one just received a diagnosis of rhabdomyosarcoma (RMS), we are sorry that you’re here, but you’ve come to the right place.
Being informed that you, your child, or someone else you love has been diagnosed with rhabdomyosarcoma (rhabdo or RMS) can be frightening, depressing, and totally overwhelming. Since it is a rare form of cancer, many pediatricians and primary care physicians may not be prepared to talk about it in any depth. It will be up to you to become as knowledgeable as you can so you can be an active advocate for yourself or your family member. FocusOnRhabdo.org is here to help as much as possible. Just knowing that you’re not alone can help ease a little of your anxiety. Join our on-line video conference support group and our Facebook Support Group and, if you can, find a local group that can assist you in coping with all that is going on.
If you haven’t already read the first section of this website “Rhabdomyosarcoma” we suggest that you do that now. Knowing a little bit about the disease is important as you will learn that two things are supremely important – the speed at which you begin treatment and the experience of those you have provided the treatment.
Do Not Dawdle… Begin Treatment Quickly!!!
Perhaps the most important advice that we personally received, was to begin treatment immediately. Don’t worry about which is the best treatment, just begin. Once treatment has begun you can continue your research and, with the help of your oncology team, adjust the treatment protocol. Rhabdo is a very fast growing cancer that metastasizes quickly and any delay in either diagnosis or treatment can greatly affect the long-term results.
Experience Counts – Get To A Sarcoma Center!
Since rhabdo is so rare, it is best treated in a Center with specialists who have dealt with this disease before. Most physicians have never seen a case of rhabdo let alone treated one.
If you cannot get the patient to a medical center with experience in treating RMS, please make sure that you take advantage of second and third opinions from the facilities and physicians who have years of experience with this rare disease. RMS is not a cancer that you can just make go-away. It is an aggressive and highly metastatic disease, which once metastasizes, less than 20% of patients are able to be cured. If you are at a ‘small’ local hospital, make sure that your doctor contacts one of the many centers that have treated rhabdo patients and insist that they work together on the proper protocol for your patient’s situation. In today’s interconnected world it should be expected that your medical records could be seamlessly shared with expert advisors around the globe.
Below is a listing of some of the major sarcoma centers around the United States. There are many mid-sized centers that also have expertise and experience in this area. We will continue to expand the listing to global centers in the coming months.
Experienced Rhabdo Centers (list to be expanded globally)
- Memorial Sloan Kettering Cancer Center – New York City
- MD Anderson Cancer Center – Houston, TX
- Los Angeles Children’s Hospital – Los Angeles CA
- St. Jude’s – Memphis, TN
- Children’s Hospital of Philadelphia – Philadelphia, PA
- Mayo Clinic – Rochester, MN
- National Cancer Institute – Bethesda, MD
- Seattle Children’s Hospital – Seattle, WA
- Stanford Children’s Health – Palo Alto, CA
- University of Texas Southwestern Medical Center – Dallas, TX
- Fred Hutchinson Cancer Research Center – Seattle, WA
- Dana-Farber Cancer Institute/Boston Children’s Hospital – Boston, MA
Read and Learn
There are two booklets available on-line that should be read. Both are written for the families of newly diagnosed rhabdo patients. The first is titled “Rhabdomyosarcoma – A Booklet for Parents. What you Need to Know. It can be found at the RhabdomyosarcomaBooklet website (http://www.rhabdomyosarcomabooklet.org.uk/). The other, “A Guide to the Sarcoma Universe For Those Who Have Just Arrived” can be found on the Liddy Shriver Sarcoma Initiative website (http://sarcomahelp.org/newly-diagnosed.html).
Staging is a way of communicating the size, aggressiveness and growth of a cancer, and is used for determining the treatment plan. It is important to understand the stage that your child’s cancer has been given so you can make better informed decisions about the treatment.
Staging rhabdomyosarcoma attempts to discover the following;
- the size of the tumor
- whether the cancer has spread to nearby lymph nodes and tissue
- whether the cancer has spread to other parts of the body
Staging is broken down into four stages and four groups. You may not be told immediately what stage or group the cancer is in because all of the diagnostic tests have yet to be completed.
- Stage 1
- The tumor is any size and has not spread to any of the lymph nodes. The tumor can be found in one of the following (only):
- Eye area
- Head and neck
- Testes, vagina, or uterus
- Stage 2
- The tumor is found in any other area not included in Stage 1 and has not spread to the lymph system. Additionally, the tumor is less than 5 cm.
- Stage 3
- The tumor is found in any area not included in Stage 1 and:
- is not larger than 5 cm and has spread to the lymph system
- is larger than 5 cm and may have spread to nearby lymph nodes.
- Stage 4
- The tumor is any size and has spread to other organs, tissues or body parts
Rhabdomyosarcoma is also grouped where the group depends on if the cancer has spread and if the cancer was able to be surgically removed.
- Group I
- The cancer was only found in one place and was removed completely during surgery.
- Group II
- The cancer is either:
- IIA – removed by surgery, but cancer was found in the margins of the tissue (the area surrounding where the tumor was removed)
- IIB – spread to nearby lymph nodes which were removed during surgery
- IIC – spread to nearby lymph nodes and cancer cells were found in the margins
- Group III
- Only some of the cancer was able to be removed through surgery or biopsy
- Group IV
- The cancer has spread to distant parts of the body from where the cancer was initially diagnosed.
Treatment of Rhabdomyosarcoma
Like many cancers, rhabdomyosarcoma is treated with chemotherapy; surgery and radiation therapy. Depending on the Stage and Group the cancer falls into will determine if one, two or all three types of treatment will be required but multi-modality therapy that includes surgery and chemotherapy with or without radiation therapy is the mainstay of treatment for RMS.
To take advantage of the latest treatments, children with this cancer should be treated at a specialized sarcoma cancer center. The doctors at these facilities have extensive experience in treating these children and have access to the latest research and protocols.
Chemotherapy uses drugs to destroy cancer cells and is given by injection into a vein (Intravenous (IV)). This is a systemic approach to treating the cancer as the drugs are injected directly into the bloodstream and reaches cancer cells wherever they may be throughout the body. The standard drugs used in North America are vincristine (Oncovin, Vincasar), dactinomycin (Cosmegen, Lyovac Cosmegen), and cyclophosphamide (Cytoxan, Clafen, Neosar). This drug combination is often referred to at VAC.
Dosages are dependent on the likelihood of recurrence:
- Low risk
- Intermediate risk
- High risk
The idea is that the intensity of the chemotherapy can be reduced if the chance of recurrence is lower.
Nearly all cases of childhood rhabdomyosarcoma are treated on clinical trials that take place through the Children’s Oncology Group (COG). A list of current clinical trials can be found on our home page.
Possible Side-Effects of Chemotherapy
The side effects of chemotherapy depend on the individual and the dose used. The side-effects can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, loss of taste buds, and diarrhea or constipation.
Here are the common side-effects of specific chemotherapy drugs:
- VAC: nausea, vomiting, and bone marrow suppression which means that the child’s immune system functioning may be reduced raising the risk of infection and other problems. Although very rare, a few patients may have significant liver damage.
- Doxorubicin: nausea, vomiting, bone marrow suppression and sores in the mouth. This drug may have long-term effect on the heart.
- Irinotecan: loose bowel movements.
- Ifosfamide and etoposide: nausea, vomiting, bone marrow suppression, and decreased kidney function.
- Temozolomide: nausea, vomiting and bone marrow suppression.
Bone Marrow Suppression (Neutropenia): Bone marrow suppression can cause a condition called neutropenia which means that there is a reduced number of neutrophils – a type of white blood cell – that helps to fight off infections by destroying harmful bacteria and fungi (yeast) that invade the body.
People that experience neutropenia have a higher risk of getting serious infections like sepsis, and anyone around or in contact with the patient must practice exceptionally good hygiene to reduce the risk of infection. This includes frequent hand washings, good dental care, avoiding any contact with sick people, cleaning cuts and bruises and covering them with a bandage. In addition to these good hygiene measures, the patients should also avoid animal waste (have someone else walk the dog or clean the cat litter), avoid unpasteurized foods, undercooked meats and raw fruits, vegetables, grains, nuts and honey, and staying out of hot tubs, ponds, rivers and oceans.
Patients find out that they have neutropenia from a blood test or when the get an infection. Excessive fatigue is one sign of neutropenia, but it is also a side-effect of most chemotherapy.
Since neutropenia is a known side-effect of chemotherapy, many doctors will prescribe an injection of Neulasta (pegfilgrastim) the day after chemo. This is a drug designed to stimulate the bone marrow into producing additional neutrophils.
It is important to understand that for a patient with neutropenia, even a minor infection can quickly become serious. Should your child exhibit any of these signs of infection, get them to a hospital without delay.
- A fever, which is a temperature of 100.5 degrees Fahrenheit or higher.
- Chills or sweating
- Sore throat, sores in the mouth, or a toothache
- Abdominal pain
- Pain near the anus
- Pain or burning when urinating, or urinating often
- Diarrhea or sores around the anus
- A cough of shortness of breath
- Any redness, swelling or pain (especially around a cut, wound, catheter, or port)
- Unusual vaginal discharge or itching
To be clear, neutropenia is dangerous and nothing to play around with. Should your child exhibit any of the above symptoms while on chemo, don’t delay a trip to the emergency room and inform the intake personnel about the low neutrophil count.
Surgery is often used as part of the treatment program for rhabdomyosarcoma. A type of surgery called wide local excision is used to remove the tumor and some of the tissue around it, including any lymph nodes that show signs of the disease. While generally a single surgical procedure is successful in removing the cancer, sometimes a second surgery is required, especially if clear margins (tissue around the tumor that does not exhibit cancer) cannot be achieved during the first procedure. Often chemotherapy is used prior to any surgery to reduce the size of the tumor. The same is true of radiation therapy. The choice of the order is dependent upon the tumor size and location.
Whether surgery is done, and which type of surgery done, depends of the following:
- Where in the body the tumor started
- The effect the surgery will have on the way the child will look
- The effect the surgery will have on the child’s important body functions
- How the tumor responded to the chemotherapy or radiation therapy that may have preceded the surgery.
Possible Side-effects of surgery:
Side-effects of surgery may include:
- Pain – it is common to have pain after any surgery. The amount and location of the pain depends on many factors;
- The location of the surgery
- The size of the incision
- The amount of tissue removed
- The amount of pain prior to surgery
Surgical pain lessens gradually as the body heals and most often, the doctor will recommend pain mediation to ease the discomfort.
- Fatigue – patients often feel very tired after major surgery, especially if the procedure involved the abdomen or chest. The fatigue may be caused by the anesthesia, the healing process, lack of nourishment caused by reduced appetite, and the stress involved with the procedure.
- Appetite loss
- Swelling around the surgical site
- Drainage from the surgical site – sometimes the fluid that builds up at the surgical site drains through the wound. Often, the surgeon will install drainage bulbs that will collect the fluid. These drains will have to be emptied on a regular basis. Drainage that smells bad, along with fever and redness around the wound are signs of infection. Should this occur, you need to contact your surgeon’s office as quickly as possible.
- Bruising around the surgical site
- Bleeding – you may experience some bleeding from the wound after surgery. If this occurs, place sterile bandages over the wound and contact your surgeon’s office. Should there be a lot of blood, apply pressure to the wound until you can get to the surgeon’s office or an emergency room.
- Infection – an infection can occur at the wound site or elsewhere in the body. Signs of infection around the incision site include redness, warmth, increased pain, and sometimes drainage from the wound site.
- Lymphedema – Lymphedema is a side-effect of surgery when multiple lymph nodes are removed. Lymph nodes are small, bean-shaped organs that help the body fight off infection. They filter bacteria and other harmful substances from the lymphatic fluid – a colorless fluid containing white blood cells that travel through most of the body’s tissues. Lymphedema occurs when, after the lymph nodes are removed, the lymphatic fluid collects in the surrounding tissues and cannot drain out causing localized swelling. Lymphedema causes swelling, discomfort and tightness in the affected area. It can limit movement and function in that area, such as an arm or a leg. If the lymphedema is severe, you might need specific therapy to manage this side-effect. This therapy is usually provided by a Certified Lymphedema Therapist (CLT).
- Organ Dysfunction – surgery in certain parts of the body, such as the abdominal cavity or chest may cause temporary problems with the surrounding organs. For example, surgery in the abdominal are can cause the intestines to become paralyzed for a short period of time. This can lead to nausea, vomiting, stomach cramps, and bloating until the bowels begin to function again. Organ dysfunction is usually a short-term side-effect and will go away as the area heals.
Radiation therapy uses high-energy x-rays or other particles (protons) to kill cancer cells and is handled by a specialist called a radiation oncologist. Most rhabdo patients are treated with external beam radiation therapy which is given by a machine while the patient is positioned on a table. Some situations call for the radiation to be delivered using implants. This is called internal radiation therapy or brachytherapy. A radiation therapy regimen usually consists of a specific number of treatments given over a specific period of time.
Usually radiation therapy is given to any area of remaining disease after 6 to 12 weeks of chemotherapy. An exception is when the tumor is near the meninges (lining of the brain), has grown into the skill bones, into the brain itself, or into the spinal cord. In these cases, radiation therapy is started right away along with the chemotherapy.
Some of the newer radiation techniques can focus more accurately on the tumor thereby reducing the exposure of nearby healthy tissue. These techniques may help increase the success rate and reduce side-effects.
- Three-dimensional conformal radiation therapy (3D-CRT) – 3D-CRT uses special computers to precisely map the location of the tumor. Depending on where the tumor is, your child may be fitted with a plastic mold resembling a body cast to keep him or her in the same position during each treatment so that the radiation can be aimed more accurately. Radiation beams are then shaped and aimed at the tumor from several directions. Each beam alone is fairly weak, which makes it less likely to damage normal body tissues, but the beams converge at the tumor to give a higher dose of radiation there.
- Intensity-modulated radiation therapy (IMRT) – IMRT is an advanced form of 3D-CRT in that along with shaping the beams and aiming them at the tumor from several angles, the intensity of the beams is adjusted to limit the dose reaching the most sensitive normal tissue. This enables the doctor deliver a higher dose to the cancer areas. Most of the major cancer centers now use IMRT.
- Proton beam therapy – Proton beam therapy differs from traditional x-ray therapy in two ways. First is that it uses a more powerful beam of protons rather than x-rays, and second, because it can be made to conform to size and shape of the cancer, thus a higher dose of radiation can be used to control and manage the cancer while significantly reducing the potential damage to the surrounding tissue.
- Brachytherapy (internal radiation therapy) – Rather than using external beams of radiation, the brachytherapy approach is to insert a radioactive source into or near the tumor for a short period of time. The radiation travels only a short distance so the tumor gets most of it. This approach may be especially useful in treating some bladder, vaginal, and head and neck tumors. Early studies suggest that this might be a way to preserve the function of these organs in many children.
Possible Side-effects of Radiation Therapy:
Side effects from radiation therapy vary depending on a few different factors including the location, number of treatments, type of radiation, depth or reach of the radiation, age of the patient, etc. Some effects last for only a short time while others might have a longer lasting impact.
Short-term side-effects include fatigue, an increased mild to severe skin reactions (radiation ‘burns’ known as radiation dermatitis), hair loss, upset stomach, and loose bowel movements. Radiation the abdominal area might cause damage to the bladder, which may cause urinary problems while radiation to the head and neck could cause mouth sores, a sore throat, and a loss of appetite. Many of these side effects go away soon after treatment is finished.
Longer-term side-effects can include the formation of scar tissue and the slowing of bone growth in areas that get radiation. Depending on the child’s age and what parts of the body get the radiation, this could result in deformities or a failure to grow to full height. Radiation can also raise the risk of cancer many years later.
Once a diagnosis has been established, the journey begins, and it is not an easy journey. Some have described it as a subterranean roller coaster ride – where the highs are much lower than you’d ever expect and the lows are pretty much down there.
Knowledge and support are critical. Check out our resource page for support groups, both online and local.
While Focus On Rhabdo cannot answer questions that are specific to a patient or a treatment plan, general questions on rhabdo can be submitted using our Contact Us form. They will be answered by a member of our Advisory Council.
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