What is rhabdomyosarcoma?
Rhabdomyosarcoma (RMS) is a rare type of cancer that forms in soft tissues, specifically skeletal muscle tissue, or sometimes hollow organs such as the bladder or uterus. RMS can occur at any age, but it affects children most often.
Although RMS can arise anywhere in the body, it is more likely to start in:
- Head and neck area
- Urinary system, such as the bladder
- Reproductive systems, such as the vagina, uterus, and testicles
- Arms and legs
The prognosis and treatment decisions depend on the type of rhabdomyosarcoma, where it starts, the size of the tumor, and whether cancer has spread. Treatment usually consists of a combination of surgery, chemotherapy, and radiation therapy.
Major advances in the treatment have significantly improved outcomes. After completing treatment, people need lifelong follow-up for possible late effects of severe chemotherapy and radiation.
Types of rhabdomyosarcoma
Rhabdomyosarcoma is a type of sarcoma (cancer) of the muscles and connective tissues of the body. There are different types. They are
- Embryonal rhabdomyosarcoma: Embryonal rhabdomyosarcoma is the most mutual kind of rhabdomyosarcoma. It can form in the head and neck area, or the genital or urinary organs.
- Alveolar rhabdomyosarcoma: Most common in adolescence, alveolar rhabdomyosarcoma occurs most often in the arms, legs, chest, abdomen, or genital area.
- Botryoid rhabdomyosarcoma: Botryoid rhabdomyosarcoma is a type of embryonal rhabdomyosarcoma and accounts for about 5-10% of all cases of rhabdomyosarcoma. It occurs most often in children between birth and 15 years.
- Pleomorphic rhabdomyosarcoma: Pleomorphic rhabdomyosarcoma generally occurs in adults.
Symptoms of rhabdomyosarcoma
The signs and symptoms depend on the location of cancer. For example, if the cancer is in the head or neck area, signs and symptoms may include, but are not limited to:
- Bulging or swelling of the eyes
- Bleeding in the nose, throat, or ears
If the cancer is in the urinary or reproductive system, signs and symptoms may include, but are not limited to:
- Difficulty urinating and blood in the urine
- Difficulty defecating
- A mass or bleeding from the vagina or rectum
If the cancer is in the arms or legs, signs and symptoms may include, but are not limited to:
- Swelling or a lump in the arm or leg
- Pain in the affected area, although sometimes there is no pain
Causes of rhabdomyosarcoma
The exact cause is not known.
Physicians know that rhabdomyosarcoma begins when a cell advances changes in its DNA. A cell’s DNA contains the instructions that tell the cell what to do. The changes tell the cell to multiply rapidly and to continue living when healthy cells would normally die. The result is a mass (tumor) of abnormal cells that can occupy and destroy healthy body tissue. Abnormal cells can separate and spread (metastasize) throughout the body.
Factors that can increase the risk of the disease include:
- Family history of cancer: The risk is higher in children with a blood relative, such as a father or brother, who has had cancer, especially if cancer started at a young age. But most children do not have a family history of cancer.
- Genetic syndromes that surge the risk of cancer: In rare cases, rhabdomyosarcoma has been related to genetic syndromes that are transmitted from parents to children, as well as neurofibromatosis 1, Noonan syndrome, Li-Fraumeni syndrome, Beckwith-Wiedemann syndrome, and Costello syndrome.
Complications and its treatment include
Cancer that spreads (metastasizes). It can spread from where it started to other areas, making treatment and recovery difficult. Rhabdomyosarcoma most often feasts to the lungs, lymph nodes, and bones.
Long-term side effects of treatment
The aggressive treatments needed to control rhabdomyosarcoma can cause substantial side effects, both short and long term. Your healthcare team can help you monitor the side effects that occur during treatment and provide you with a list of side effects to watch for in the years after treatment.
Diagnosis of rhabdomyosarcoma
The diagnosis of rhabdomyosarcoma usually begins with a physical exam to better understand the symptoms that you or your child may be experiencing. Based on those findings, other tests and procedures may be recommended.
Your doctor may recommend one or more imaging tests to look for symptoms, look for cancer, and look for signs that cancer has spread.
Imaging tests may include:
- Computed tomography (CT)
- Magnetic resonance imaging (MRI)
- Positron emission tomography (PET)
- Bone scan
Removing a sample of tissue for analysis (biopsy). A biopsy process is used to collect a sample of doubtful cells for laboratory testing. The tests can show if the cells are cancerous and determine the type of cancer.
The types of biopsy procedures used to diagnose rhabdomyosarcoma include:
- Needle biopsy: The doctor inserts a fine needle through the skin and guides it to the tumor. The needle is used to remove unimportant pieces of tissue from the tumor.
- Surgical biopsy: The doctor makes an incision through the skin and removes the entire tumor (excisional biopsy) or part of the tumor (incisional biopsy).
Determining the type of biopsy needed and the details of how it should be performed requires careful planning on the part of the medical team. Doctors must perform the biopsy in a way that does not interfere with future surgery to remove cancer. For this reason, before your biopsy, ask your doctor for a referral to a team of experts with extensive experience treating rhabdomyosarcoma.
Treatment of RMS generally involves a combination of treatments, including chemotherapy, surgery, and radiation therapy.
The treatments your physician mentions will depend on the location of cancer, the size of the tumor, whether the cells are aggressive, and whether the cancer is confined to one area (localized rhabdomyosarcoma) or has spread to other areas of the body (metastatic rhabdomyosarcoma).
The goal of surgery is to kill all cancer cells. But it is not always possible to do this if the RMS has grown around or near organs or other important structures. When cancer cannot be completely removed with surgery, doctors can remove as much of it as possible and then use other treatments, such as chemotherapy and radiation, to kill any cancer cells that may remain.
Chemotherapy uses powerful drugs to kill cancer cells. Treatment usually involves a combination of medications, which are usually given through a vein. Which medications are given and how often will vary depending on your particular situation.
Chemotherapy is often used after surgery or radiation therapy to kill any cancer cells that may remain. It can also be used before other treatments to shrink a tumor and make surgery or radiation therapy more effective.
Radiation therapy uses high-energy rays, such as x-rays and protons, to kill cancer cells. It is usually done with a machine that moves around you while directing radiation to precise points on the body.
Radiation therapy may be recommended after surgery to kill any remaining cancer cells. It can also be used in place of surgery when the RMS is located in an area where surgery is not likely due to nearby organs or other important buildings.
While it is always good to maintain a healthy weight and quit smoking or using tobacco, there are currently no known lifestyle changes that can prevent bone cancer.