Rhabdomyosarcoma - Cause, Types, Symptoms, Diagnosis, Complications and Treatment

What Is Rhabdomyosarcoma?

Rhabdomyosarcoma is a rare cancer in the body's soft tissues, usually the striated muscles (skeletal muscles). The skeletal muscles are the muscles that are used to move the parts of our body. Rhabdomyosarcoma can occur at any age, but it commonly affects children. It can occur anywhere in the body but is more widely seen in the head and neck region, arms, legs, the urinary system like the bladder, and reproductive systems like the uterus, vagina, and testes.

What Is the Cause for Rhabdomyosarcoma?

Rhabdomyosarcoma develops from the mesenchymal cells that have failed to differentiate into myocytes of skeletal muscle completely. Cells of the tumor are recognized as rhabdomyoblasts. The exact cause of rhabdomyosarcoma is not known, and it is not easy to diagnose. It is also associated with specific genetic mutations and chromosomal abnormalities.

The inherited conditions which increase the risk of acquiring rhabdomyosarcoma are:

• Beckwith-Wiedemann syndrome.

• Li-Fraumeni syndrome.

• Neurofibromatosis type 1.

• Noonan syndrome.

• Costello syndrome.

• DICER1 syndrome.

What are the Types of Rhabdomyosarcoma?

There are four types of rhabdomyosarcoma which include:

1. Embryonal: It is the most common histological variant that comprises 60 % to 70 % of cases in children. It is most commonly seen in children up to four years of age. These tumors are usually seen in the head, neck, and genitourinary tract.

2. Alveolar: It is the second most common type, and it comprises 20 to 25 % of rhabdomyosarcoma-related cases in children. It is commonly seen in young adults and teenagers. They usually occur in the trunk, extremities, and peritoneum. It is more aggressive than embryonal rhabdomyosarcoma.

3. Pleomorphic: It is also called anaplastic rhabdomyosarcoma. It is usually seen in adults and rarely in children.

4. Spindle Cell or Sclerosing: It is the cancer of the smooth muscle tissue. It is most commonly seen in the paratesticular regions. The prognosis is excellent for this type of rhabdomyosarcoma.

What Are the Signs and Symptoms of Rhabdomyosarcoma?

The symptoms usually depend upon the location of the tumor they include:

• Presence of a painless lump.

• Bulging or swelling of the eyes.

• Hematuria.

• Abdominal pain.

• Bleeding in the nose, throat, rectum, or vagina if the tumor is around these organs.

• Numbness, tingling, and pain in case of nerve compression.

• Congestion and difficulty in swallowing if the cancer is present near the throat and nose.

• The vaginal tumor will visibly protrude.

• Headache.

• Blood in the urine or difficulty in urinating due to urinary tract obstruction.

• Difficulty in bowel movements.

• Cranial nerve dysfunction.

• Sinusitis.

• Ear discharge.

• Facial pain.

How to Diagnose Rhabdomyosarcoma?

1. Physical examination:

Rhabdomyosarcoma usually begins with a physical examination to understand the symptoms of the patient. Based on the findings in the examination, the other tests and procedures are performed. The doctor can recommend one or more imaging tests.

2. Imaging Tests:

• CT (computed tomography) scan - This is a combination of x-ray and computer technology that produces images of the body sections such as fats, bones, organs, and muscles.

• MRI (magnetic resonance imaging) - It will provide detailed images of the organs and the structures.

• X-ray - It produces images of the bones, organs, and internal tissues onto the x-ray film.

• Positron Emission Tomography (PET) - Radiotracers are used to perform imaging tests to assess the tissue and organ functions.

• Bone Scan - X-rays of the bone are taken after the dye injection, which will locate bone tumors and abnormalities.

• Ultrasound - High-frequency sound waves are used to produce images of the blood vessels, tissues, and organs.

3. Biopsy: A sample of tissue is removed from the tumor to be tested under a microscope.

• Needle Biopsy - This is one type of biopsy used to remove tissue and test for rhabdomyosarcoma. In this procedure, the doctor inserts the needle into the skin and guides it into the tumor. This thin needle will remove small pieces of tissues from the tumor.

• Surgical Biopsy - In this procedure, the doctor will make an incision in the skin and will remove either the entire tumor (excisional biopsy) or the small portion of the tumor (incisional biopsy).

How to Treat Rhabdomyosarcoma?

The main goal of treatment is to remove the cancer cells and maintain the function and minimize the disability. The treatment depends on the location and size of the tumor, the patient's age, and the patient's capability to tolerate the therapies, many of which can have severe side effects. Treatment typically involves some combination of surgery, chemotherapy, and radiation therapy. The surgeon eliminates as much of the tumor as possible. The doctor will try to reduce the damage or disfigurement when doing the procedure, but that can be not easy.

1. Surgery: The main goal of surgery is to eliminate cancer cells. But it is not possible to do that if the rhabdomyosarcoma has developed near or around organs or other vital structures. When the doctor cannot eradicate cancer with surgery, they may remove as much as possible and then use other methods, such as chemotherapy and radiation therapy, to kill any cancer cells that have been left.

2. Chemotherapy: Chemotherapy uses potent drugs to kill cancer cells. The treatment involves the introduction of a combination of drugs, which are commonly administered through a vein. Types of drugs and how often it is given vary depending on the particular situation. Chemotherapy is usually done after surgery or radiation therapy to kill any cancer cells that remain after those procedures. It is also used to contract or shrink a tumor to make surgery or radiation therapy more effective.

3. Radiation therapy: Radiation therapy employs high-energy beams, such as X-rays and protons, to kill cancer cells. It is done with a device that moves around you as it directs the radiation at precise points on the body. Radiation therapy is recommended after surgery to kill any cancer cells that are left. It can also be used alternatively for surgery when the rhabdomyosarcoma is located in an area where surgery is impossible because of nearby organs or other vital structures. Brachytherapy, or placing small, radioactive seeds directly inside the tumor or cancer site, is often indicated in children with tumors of delicate areas such as the testicles, vagina, or bladder. This decreases scattering and the development of late toxicity following dosing.

4. Immunotherapy:

Immunotherapy is a more modern treatment modality. This method involves selecting and preparing the patient's immune system to target the cancer cells. We can accomplish this by administering small molecules created to pull immune cells towards the tumors, take immune cells drawn from the patient, and train them to attack tumors through remembrance with tumor antigens or other innovative methods.

What Are the Complications of Rhabdomyosarcoma?

The complications of rhabdomyosarcoma may include:

• Bladder damage.

• Infertility caused by chemotherapeutic agents.

• Secondary cancer may occur after treatment.

• Rhabdomyosarcoma can spread to other areas from where it started, making the treatment and the recovery very difficult.

• It can spread to the organs like lymph nodes, lungs, and bones.

• Side effects can be caused due to the aggressive treatment given to control rhabdomyosarcoma symptoms in long and short-term therapy.

Conclusion

Many cancers in adults can be prevented by lifestyle changes like quitting smoking, using tobacco, and maintaining a healthy weight. Still, there are no known lifestyle changes to avoid cancers in children. The known cause of rhabdomyosarcoma is genetics, age, and gender, which cannot be changed; therefore, there are no proven environmental or lifestyle-related causes for rhabdomyosarcoma. You can get more information regarding this disease through online consultation also.

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