Rhabdomyosarcoma (RMS)
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Rhabdomyosarcoma (RMS) is a rare type of cancer that develops in the muscle tissue, or it could also affect hollow organs such as the bladder or uterus. RMS can affect people at any age, but children and adolescents are the most common.
In rhabdomyosarcoma, mesenchymal cells, which are responsible to form cells into muscles, change and multiply out of control, resulting in the growth of tumors.
Considerable improvements in rhabdomyosarcoma outcomes have been made possible by advances in treatment. Treatment options are surgery, chemotherapy and radiation therapy depending on the cancer’s type, size, location, and if the cancer has spread.
RMS can develop anywhere in the body; however, it typically begins in the:
- Head and neck area: near the spine in the neck, inside the nasal sinuses or the throat, or close to the eye.
- Urinary system: such as bladder
- Reproductive system: such as the vagina, uterus, and testes
- Arms and legs
Types
These are the types of rhabdomyosarcoma:
- Embryonal rhabdomyosarcoma: The most typical type of rhabdomyosarcoma, which usually affects the reproductive and urinary systems or the head and neck.
- Botryoid rhabdomyosarcoma: A subtype of embryonal rhabdomyosarcoma. It is most frequently found in hollow organs such as bladder, vagina.
- Spindle rhabdomyosarcoma: A subtype of embryonal rhabdomyosarcoma. It commonly appears near at the boy’s testicles.
- Alveolar rhabdomyosarcoma: Aggressive type that typically found in the arms, legs, or torso.
- Pleomorphic and undifferentiated rhabdomyosarcoma: A rare type of rhabdomyosarcoma. It affects the arms, legs, or torso of children.
Symptoms
Rhabdomyosarcoma symptoms and signs vary depending on the location of the tumor.
- Head or neck area:
- Headache
- Eye swelling
- Bleeding in the nose, throat or ears
- Urinary or reproductive system:
- Difficulty in urinating or blood in the urine
- Difficulty with bowel movements
- Vaginal bleeding or rectal bleeding
- Lump in the vagina or rectum
- Arms or legs:
- Swelling in the arm or leg
- Lump in the arm or leg
- Pain in the arm or leg.
Causes
The exact cause of rhabdomyosarcoma is still unknown. Rhabdomyosarcoma is known to start when the DNA of the healthy cells mutate causing uncontrolled duplication, resulting in forming a mass of cells also known as a tumor. This tumor then starts to invade and destroy the healthy tissue and could metastasize (spread to other parts of the body).
Risk factors
The following factors may increase the risk to develop rhabdomyosarcoma:
- Age and gender: RMS typically develops in children under the age of 10, however it can also occur in adults and teenagers. Males are more likely to develop it than females.
- Family history: Children who have a family member (parents or siblings) who had rhabdomyosarcoma are more at risk to develop the disease, especially if that family member was diagnosed at an early age. However, the majority of children with rhabdomyosarcoma do not have any history of cancer in the family.
- Genetic syndromes. In rare cases, genetic disorders transferred from parents to children have been associated to rhabdomyosarcoma:
- Neurofibromatosis 1: it frequently results in many nerve tumors (particularly in the skin’s nerves), but it also raises the risk of RMS. Referred to as von R
ecklinghausen disease - Noonan syndrome: Children with this disease suffer cardiac problems, tend to be short, and have delay development. They are also likely to develop RMS.
- Li-Fraumeni syndrome: Family members with this condition are more prone to develop sarcomas (including RMS), breast cancer, leukemia, and some other cancers.
- Beckwith-Wiedemann syndrome: Children with Beckwith-Wiedemann syndrome are more prone to get RMS and several other pediatric cancers.
- Costello syndrome: Children with this syndrome are short, have a high birth weight and typically have a large head. They are more likely to get RMS and several other types of malignancies.
- Neurofibromatosis 1: it frequently results in many nerve tumors (particularly in the skin’s nerves), but it also raises the risk of RMS. Referred to as von R
Diagnosis
Physical examinations are used to diagnose rhabdomyosarcoma in order to investigate any symptoms that the patient has. More tests and procedures will be recommended based on the result of the physical examination.
- Imaging tests: In order to examine your symptoms, rule out cancer, and check for signs of the disease spreading, your doctor can advise one or more imaging tests, which includes:
- X-ray: While X-rays are sometimes used to look for malignancies, they are most effective when utilized to examine bones.
- Computerized tomography (CT): To provide comprehensive cross-sectional images of body parts, a CT scan combines a number of x-ray images. To observe details better during the scan, a
contrast agent may be injected into a vein beforehand. CT scan can provide a clear image of the size of the tumor and its surrounding tissues. It can also be used to examine surrounding lymph nodes, the lungs, or other parts of the body where the cancer may have spread. - Magnetic resonance imaging (MRI): Instead of using x-rays, MRI scans use radio waves and powerful magnets to produce detailed images. In order to observe details more clearly during the scan, gadolinium (contrast agent) may be injected into a vein beforehand. MRI can be utilized if the tumor is located at a specific part of the body, such as the head and neck, arm or leg, or at the pelvis. MRI could also show the exact size of the tumor
- Positron emission tomography (PET): A radioactive material is injected into the blood for a PET scan. The locations of radioactivity in the entire body can then be captured on film using a specialized camera.
- Bone scan: A bone scan can determine whether a malignancy has metastasized to the bones as it gives a comprehensive image of the complete skeleton.
- Biopsy: A sample of suspected cells is taken during a biopsy process and tested in a lab. Tests can identify the type of cancer and reveal whether the cells are malignant. The following types of biopsy techniques are used to identify rhabdomyosarcoma:
- Needle biopsy: A tiny needle is inserted into the tumor by the doctor through the patient’s skin. A tissue sample is taken out from the tumor with the needle.
- Surgical biopsy: Through a skin incision, the
physician removes the tumor as a whole (excisional biopsy) or a portion of it (incisional biopsy). - Bone marrow aspiration and biopsy: During a bone marrow biopsy, a sample of bone, blood, and bone marrow are typically taken from one or both hip bones. These tests are frequently performed following the diagnosis of RMS to determine whether the malignancy has progressed to the bone marrow.
- Lumbar puncture: This procedure, also known as a spinal tap, involves drawing fluid from the spine with a needle. Although not frequently performed for RMS, this test may be carried out if a tumor is present close to the covering of the brain (the meninges). The cerebrospinal fluid (CSF), the fluid that surrounds the brain and spinal cord, is tested for the presence of cancer cells.
Treatment
Chemotherapy, surgery, and radiation therapy are frequently used in the treatment of rhabdomyosarcoma. The course of treatment will depend on the cancer’s location, the size of the tumor, and if it has spread to other parts of the body.
- Surgery: The purpose of surgery is to eliminate all cancer cells. However, if the rhabdomyosarcoma has spread close to or around vital organs or other significant structures, it may not always be possible to do that. When surgery cannot entirely remove the cancer, the doctors may remove as much of it as they are able before using alternative therapies to remove any remaining cancer cells, such as chemotherapy and radiation.
- Chemotherapy: Chemotherapy kills cancer cells by using medications. Drugs are typically given in
combination as part of the treatment, typically through a vein. The medications administered and their frequency depends on the circumstances. After surgery or radiation therapy, chemotherapy is frequently used to eliminate any cancer cells that could have remained. Additionally, it can be used to reduce a tumor before additional therapies to improve the effectiveness of surgery or radiation therapy. Please revise this. - Radiation therapy: High-energy beams, including X-rays and protons, are used in radiation therapy to kill cancer cells. The radiation is frequently directed to specific locations on the body using a machine. After surgery, radiation therapy could be advised to eliminate any cancer cells that survived. Additionally, it can be utilized in place of surgery when the rhabdomyosarcoma is located in an area where it is impossible to do surgery because to the proximity of vital organs or other structures.
