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rhabdomyosarcoma

About Rhabdomyosarcoma
Rhabdomyosarcoma is a type of soft tissue sarcomas that start from skeletal muscles. Sarcomas are cancers that begin from connective and supportive tissues in the body. The main two types of sarcomas are soft tissues and bone sarcomas. Soft tissue sarcomas include those that develop in muscles, fat, blood vessels and nerve tissues. Muscles as one of the soft tissues is further divided into three types;
• Smooth muscles. This is type of muscle is found to those internal organs. For example those muscles in intestines that help to move food in the digestive system.
• Skeletal muscles. These are the ones that help us to move.
• Cardiac muscles. These are those found in the heart. These enabled the heart to pump blood.
Rhabdomyosarcoma starts from skeletal muscles. It is form of cancer which is more common to children and young adults although it can sometimes develop to adults. Rhabdomyosarcoma develop from immature cells (mesenchymal cells) that develop to form muscles.
We might think of our skeletal muscles as being mainly in our arms and legs, but these skeletal muscle cancers can start nearly anywhere in the body, even in some parts of the body that don’t normally have skeletal muscle. Common sites for rhabdomyosarcoma include head and neck (skull, nasal cavity, eye socket, throat and spine), arms, legs, trunk (chest and abdomen), prostate, urinary bladder and areas around testicles. Rhabdomyosarcoma is divided into main three types;
• Embryonal rhabdomyosarcoma. This is the most common form of rhabdomyosarcoma to young children. It is more common to those below 6 years. Embryonal rhabdomyosarcoma is further subdivided into two types.
• Botryoid rhabdomyosarcoma. Is a subtype of embryonal rhabdomyosarcoma that is mostly found in the walls of hollow, mucosa lined structures such as urinary bladder, vagina and common bile duct.
• Spindle cell rhabdomyosarcoma. It is a subtype of embryonal rhabdomyosarcoma that commonly occurs in areas around testicles (paratesticular soft tissues).
• Alveolar rhabdomyosarcoma. This is the type of rhabdomyosarcoma that tend to occur in older children and adolescents. This is more aggressive form of rhabdomyosarcoma compared to embryonal rhabdomyosarcoma. It is normally found in the arms, legs or trunk (chest and abdomen).
• Anaplastic (pleomorphic) rhabdomyosarcoma. This is uncommon type of rhabdomyosarcoma which occurs in adults but it is rare to children. It normally develops in arms, legs or trunk. This type of cancer grows quickly and requires intensive treatment.
Because rhabdomyosarcoma is more common to children compared to adults, most of the information in this document will focus to children although they can be also be applied to adults.

Risk factors of rhabdomyosarcoma
Risk factor is anything that increase a person`s chance to develop the disease (cancer). For adult cancers, lifestyle and environmental factors (such as smoking or exposure to certain chemicals) can be significant risk factors for developing certain types of cancer. But these factors usually take many years to influence cancer risk, and they are not thought to play much of a role in childhood cancers, including rhabdomyosarcoma. For most children with cancer, the underlying cause is unknown.
Even if your child has a risk factor, it does not mean they will develop cancer. Many children with a risk factor will never develop cancer, while others with cancer may have had no known risk factors. Even if a child with a risk factor develops cancer, it is usually hard to know how much that risk factor contributed to the development of their disease. Below are some of the factors that may increase the risk of developing rhabdomyosarcoma.
• Age. Although retinoblastoma can develop at any age, children below 10 years are said to be at an increased risk of developing the disease as statistics shows that, most cases of rhabdomyosarcoma are from that age group.
• Hereditary conditions. There are some hereditary conditions that are said to increase the possibility of developing rhabdomyosarcoma to someone with any of those conditions. These conditions include;
• Li-fraumeni syndrome. This is the condition which is associated with mutation of TP53 gene which is a blueprint for a protein called p53. This p53 is a tumor suppressor gene which regulate cell cycle and prevent development of cancer. Mutation of this gene normally increase the risk of breast cancer, leukemia, sarcomas (including rhabdomyosarcoma) and other cancers.
• Neurofibromatosis type 1. This is a human disorder caused by mutation of gene that is responsible for production of protein called Neurofibromin. It usually causes tumors to grow along the nerves. It also increases the risk of developing rhabdomyosarcoma.
• Beckwith – wiedemann syndrome. This is an overgrowth disorder which is usually present at birth. Children with this condition are said to be larger than normal. This condition increases risk of childhood cancers including rhabdomyosarcoma and wilm`s tumors.
• Costello syndrome. Children with this syndrome have high birth weights but then fail to grow well and are short. They also tend to have a large head. These children have an increased risk of developing some cancers including rhabdomyosarcomas.

Prevention of rhabdomyosarcoma
To prevent something you need first to know what causes it. Unfortunately there are no known causes for rhabdomyosarcoma so currently there are no ways to prevent this type of cancer. The difference between adult cancers and children cancers is that, adult cancers are highly associated with life style and environmental factors such as smoking and obesity, so by controlling those factors it may reduce the risk of developing those cancers.
But when it comes to these children cancers, lifestyle and environmental factors are believed not to be the reason behind development of cancers, so currently there are no ways to even reduce the risk of developing cancer to children.

Early detection and screening
When it comes to cancer, early detection normally provide better treatment outcome. When rhabdomyosarcoma is found at an early stage, chance for better treatment outcome is very high. For several types of cancers, screening has helped in their early detection.
Screening is the process of running some tests to someone with no signs or symptoms of a certain disease with the intention of determining presence or absence of the disease of that disease. However currently there are no screening tests recommended for rhabdomyosarcoma. Still, rhabdomyosarcoma often causes symptoms that allows it to be found early before it has spread to other parts of the body.

Signs and symptoms of rhabdomyosarcoma
Children with rhabdomyosarcoma may experience (have) some of the following signs and symptoms. Sometimes those signs and symptoms may be caused by other medical conditions other than rhabdomyosarcoma, so the only way you can find out is through seeing the doctor for more examination as soon as you realize that your child has one or some of those signs and symptoms.
Signs and symptoms of rhabdomyosarcoma depends on the where tumor is, how large it is and if tumor has spread to other parts of the body. In most cases, rhabdomyosarcoma occurs in areas where the symptoms of the disease are easily noticeable which prompt their early detection. Among those signs and symptoms include the following;
• A tumor in the abdomen can cause abdominal pain and constipation.
• A tumor in the head and neck can cause blockage (obstruction) and/or discharge from nose, throat and ears. Eyes may appear swollen and protruding.
• Tumors in the pelvis (bladder, vagina and prostate) can cause blood in the urine, vagina bleeding, feeling pain to urinate and difficult bowel movement.

Diagnosis of rhabdomyosarcoma
Following suspicion of rhabdomyosarcoma, doctors use various tests to locate the disease, learn if the disease has spread to other organs of the body and some tests will help to determine stage of the disease. Before or during running those diagnostic tests to your child, among the things that your doctor will consider include the following;
• Results of previous tests
• General health condition of your child
• Type of cancer suspected
• Age
The following are some of the diagnostic tests that can be used for diagnosis of rhabdomyosarcoma;
• Physical examination and medical history. Although there will be suspicion that your child has rhabdomyosarcoma, the doctor would want to know more about the symptoms and for how long has your child experienced them so as to plan for further diagnostic tests. Also your doctor will examine your child to look for anything abnormal. For example presence of lump or swelling.
• X-rays. Although their use when dealing with rhabdomyosarcoma is limited, plane x-rays will help to show if tumor has spread to the bones. Plane x-rays are not good for examination of soft tissues but good for examination of hard ones (bones).
• CT scan. Computed tomography is a diagnostic test that uses x-rays to generate images of different organs of the body. This test is different from the plane x-ray imaging as CT uses x-rays to create many pictures of body parts from different angles, then computer combine these images to produce a detailed cross sectional images of your body parts.
CT scan is used to locate the tumor and show if tumor has spread to other parts of the body. Before scanning, you may be given a contrast that will make the images to be look clear. This can be given orally or through injection (intravenously).
• MRI scan. Magnetic resonance imaging uses radio waves and strong magnetic field to generate picture of internal parts of the body. Magnetic resonance imaging is used to locate tumor and show if cancer has spread to other parts of the body.
Unlike CT scan, MRI uses magnetic field and radio waves to create images of body parts. Normally, either a CT or an MRI scan is needed, but usually not both. Because CT scans give off radiation, which might raise a child’s risk for other cancers in the future, most doctors prefer to use MRI.
• Bone scan. Bone scan Bone scan is a nuclear medicine test that uses very small amount of radioactive substance called tracer. This is used to examine if retinoblastoma has spread to the bones. During the test, small amount of radioactive tracer will be injected (intravenously), where the tracer will settle in high concentration to the bones with abnormality throughout the body. Special camera will be used to detect the radioactivity and show picture of the skeleton.
• Biopsy. Biopsy is a diagnostic test that involve removing small tissue sample for examination under microscope in the laboratory. Examination is done by the pathologist (a doctor specialized in interpreting and evaluating samples in the laboratory). This is used as confirmatory test, as other imaging tests can only locate the tumor but cannot give more details such as type of cancer.
• Immunohistochemistry. This involve examination of special stains on the cells taken during the biopsy to help the doctor make an accurate diagnosis of rhabdomyosarcoma. The strains show muscle cell development, including actin and desmin.

Staging of rhabdomyosarcoma
Staging is a standard way used by cancer care team to explain the location of tumor, size of the tumor and extent of spread of cancer. Staging also enables the oncology team to decide on the suitable treatment option. In this process of staging doctors normally use systems in order to describe the stage of cancers. When dealing with rhabdomyosarcoma, staging is a bit different from how other cancers are staged. Stages of rhabdomyosarcoma are described as follows;
TNM staging system
TNM staging system is the combination of tumor, node and metastasis.
• Tumor (T). This describe the location and size of tumor.
• Node (N). This describe lymph node involvement
• Metastasis (M). This describe the spread of tumor to other organs.
To describe the stage of the disease, these three terms are combined together to give a detailed information about the tumor.
• Stage 1. Tumor is of any size but it should be located in the orbit, head and neck except for parameningeal (areas near the membranes covering the brain such as nasal sinuses, middle eye and upper part of throat), bile duct and genital urinary tract except for prostate and bladder. There is no lymph node involvement and distant metastasis.
• Stage 2. Tumor is smaller than 5cm and it is located in any part of the body not mentioned in stage 1 such as bladder, prostate, parameningeal, arms and legs. There is no evidence of lymph node involvement or distant metastasis.
• Stage 3. Tumor is located in any part of the body not mentioned in stage 1 such as bladder, prostate, parameningeal, arms and legs. Tumor is smaller than 5cm but has spread to nearby lymph node or tumor is larger than 5cm but may/may not have spread to nearby lymph nodes. There is no distant metastasis.
• Stage 4. Tumor is of any size and it is located anywhere in the body but there should be distant metastasis.

Clinical groups
This describe tumor by considering whether surgeon can remove tumor completely or not.
• Group I. Tumor can completely be removed by surgery.
• Group II. Tumor has been removed by surgery but there are remains of cancer cells at the edge of tissues that surrounded the tumor (margins). Cancer cells may also be found in nearby lymph nodes.
• Group III. Tumor is located in its original area (it has not spread to distant sites), but it cannot be removed by surgery.
• Group IV. Tumor has spread to other distant parts of the body.

Treatment of rhabdomyosarcoma
Children cancers are not common, so even their management is normally slightly different from the way adult cancers are managed. This is way children cancers are treated in a specialized centers that deals with management of children cancers.
Children with cancers and their families have special needs and these needs can be met best by children`s cancer centers, working closely with the child’s primary care doctor. These centers have teams of specialists who understand the differences between cancers in adults and those in children, as well as the unique needs of younger people with cancer.

Making treatment decision
If a person with cancer is a child then treatment decision has to be made by the parents. With the help of a child`s doctor, parents have to take time to understand about the condition their child, treatment options and possible outcome of treatment. Parents are free to ask if there is anything they do not understand clearly for example any possible side effects of treatments.
Normally when making treatment plan, when it comes to treatment options, doctors normally provide treatment options basing on the following;
• Stage of the disease
• General health condition of a child
• Possible side effects
• Parent`s preference

Taking part in clinical trial
Clinical trials are new drugs or modified mode of treatment or new treatment combination for cases like yours that are to be tested if they are effective to be used as standard care treatment option or not. Your child`s doctor or nurse will discuss with you if you want your child to take part in clinical trial.
Over the years, trials have improved treatments and led to better outcomes to children cancers. It may be helpful to talk with another specialist to get second opinion before letting your child take part in clinical trials. And if you decide to take part, you can also withdraw at any time.

Treatment options
Treatment of rhabdomyosarcoma requires team of doctors (pediatric oncologists, orthopedic surgeon and radiation oncologist), nurses and other health care professionals. This team is called multidisciplinary team. In some cases, when tumor is in head and neck, surgery may require ENT surgeons (ear, nose and throat), neurosurgeons, maxillofacial surgeon and plastic surgeon. Also pediatric cancer centers normally have additional support services for children and their families, such as child life specialists, dietitians, physical and occupational therapists, social workers, and counselors. Treatment options of rhabdomyosarcoma include the following
Surgery
Surgery is the treatment option that include removal of tumor and some surrounding health tissues by operation. This treatment option is delivered by orthopedic surgeon. When dealing with rhabdomyosarcoma, surgery is preferred when tumor is small and can completely be removed leaving cancer free margins.
In some cases, surgery may be done even if it is clear that all of the cancer can’t be completely removed. Surgery will be done because it may help other treatments (chemotherapy and radiation) to work better.
If tumor is large or it is located in a spot where surgery cannot be done as it would result into other serious effects, then surgery will be delayed until after some course of chemotherapy or radiotherapy to shrink the tumor or surgery will not be done at all.

Side effects of surgery
Side effects of surgery will depend on the location of tumor, type and extent of surgery. Possible side effects of surgery include short term effects and long term effects. These side effects include,
• Bleeding
• Reaction from anesthesia
• Pain
• Risk of infection
Long term side effects will mostly depend on extent of surgery as these include changing person`s appearance or how some parts of the body work.

Chemotherapy
Chemotherapy is the use of anti-cancer drugs to kill, destroy or inhibit growth of cancer cells. Chemotherapy is delivered by pediatric oncologist (medical oncologist). Chemotherapy can be given through injection (intravenously) or as pill or capsule which is swallowed orally.
Chemotherapy is sometimes given before surgery so as to shrink the tumor or after surgery so as destroy any possible remains of cancer cells in order to reduce the risk of rhabdomyosarcoma coming back.

Side effects of chemotherapy
Side effects of rhabdomyosarcoma vary depending on the type of drugs, dose given and for how long the drug has been used. Some of the side effects will go away on their own while some will require to be relieved by medications. Your treatment team will be there to assist you with those side effects. Possible side effects of chemotherapy include;
• Hair loss
• Mouth sores
• Nausea and/or vomiting
• Diarrhea
• Risk of infections due to decreased white blood cells
• Fatigue as a result of low red blood cells
• Easily bruised and bleeding as a result of low platelets.

Radiation therapy
Radiation therapy is the use of high energy x-rays or other radiation particles to destroy cancer cells. Radiation therapy is given by a specialized doctor known as radiation oncologist. When dealing with rhabdomyosarcoma, radiotherapy is normally given in two ways;
• External beam radiation therapy. Radiations are directed to the tumor from the source (machine) position outside the body.
• Brachytherapy. This is also called internal radiation therapy. In this, source of radiation is inserted into the tumor or near the tumor. The source is called an implant and it will be left there to deliver radiation for some time (short period). This technique will reduce the risk of exposing other normal organs.

Side effects of radiation therapy
Side effects of radiation therapy will depend on site of irradiation, technique used and dose of radiations. There are side effects that will last for short time but also there may be those side effects that may have longer lasting impact. The side effects include the following;
• Tiredness
• Skin reactions
• Nausea and/or vomiting
• Diarrhea
• Mouth sores (radiation to the head)
Long term possible side effects include;
• Memory loss and change of personality. These may be the result of irradiation of brain
• Effects to bone growth. When bones are irradiated, their growth may be affected resulting to deformities or not growing to full height.

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