Is Rhabdomyosarcoma Soft Tissue Sarcoma?

Medically Reviewed on 4/20/2022
Is rhabdomyosarcoma soft tissue sarcoma?
Rhabdomyosarcoma is a type of sarcoma made up of tumors that arise from muscle tissue and spread throughout the body.

The most common kind of soft tissue sarcoma in children is rhabdomyosarcoma. Overall, it is still a rare malignancy, accounting for about three percent of all childhood malignancies. These tumors arise from muscle tissue and can spread throughout the body.

Every year, 400 to 500 new cases of rhabdomyosarcoma are detected in the United States.

2 major forms of rhabdomyosarcomas

  1. Embryonal rhabdomyosarcoma
    • This form is more common in the:
      • Head
      • Neck
      • Bladder
      • Vagina
      • Prostate
      • Testes
    • Infants and young children are mostly affected by this type although it can occur later as well.
    • It is the most common type of rhabdomyosarcoma.
  2. Alveolar rhabdomyosarcoma
    • This kind of rhabdomyosarcoma is more common in the:
      • Large muscles of the trunk
      • Arms
      • Legs
    • It more commonly affects older children or teenagers and adults.
    • The term “alveolar” refers to cancer cells that develop little hollow pockets or “alveoli.”

Rhabdomyosarcoma is most usually found in the head and neck, bladder, testes, womb, or vagina. Tumors can be found in the leg, chest, or abdominal wall. If the tumor is in the head or neck, it has the potential to spread into the brain or the cerebrospinal fluid (CSF).

7 signs and symptoms of rhabdomyosarcoma

The symptoms of rhabdomyosarcoma (RMS) might differ greatly depending on where the tumor develops.

  1. Children with orbital RMS (which accounts for about 10 percent of all RMS cases) may present with a bulging or enlarged eye (proptosis).
  2. Children with tumors in one of the parameningeal sites (essentially the sinuses, middle ear, and uppermost part of the throat) may have a stuffy nose for weeks or months and sometimes present with nasal discharge. Occasionally, a mass may be visible in the nostril or the back of the throat.
  3. If the tumor erodes at the skull base, they may experience headaches or develop cranial neuropathies due to infiltration or compression of affected cranial nerves.
  4. Children with genitourinary tract cancers may manifest with a painless scrotal lump (paratesticular tumors), a projecting grape-like mass in the vagina (“botryoid” rhabdomyosarcoma), blood in the urine (bladder tumors), or frequent urination, often with a burning sensation or hesitation.
  5. Tumors in the prostate gland can become quite large before they are discovered. These tumors may show as a palpable mass in the pelvis or abdomen. They may sometimes have urine frequency and urgency, constipation, nausea, or vomiting from intestinal compression.
  6. Tumors that develop in the legs or arms are typically among the more aggressive kinds of RMS. In a few weeks, these tumors can develop from the size of a mosquito bite or little marble to the size of a baseball or grapefruit. Tumors are usually hard. They are rarely painful unless they impinge on adjacent nerves. These tumors are the most likely to spread to the neighboring lymph nodes. It is common for a kid with RMS in the hand, arm, foot, or calf to have swollen glands in the armpit or groin.
  7. Children with RMS may experience unexplained fevers as one of their symptoms at the time of diagnosis. Appetite may or may not be suppressed. Unless the tumor has migrated to the bone marrow, fatigue and easy bruising are uncommon signs.

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3 possible causes and risk factors of rhabdomyosarcoma

The exact cause of rhabdomyosarcoma (RMS) is unknown. Studies suggest that it results due to some abnormal change or mutation in the DNA. This results in the formation of abnormal cells that multiply uncontrollably forming a tumor.

Although the specific risk factors for rhabdomyosarcoma are unknown, there is an increased risk of rhabdomyosarcoma due to radiation exposure or the use of certain recreational drugs by the mother during pregnancy and certain genetic conditions.

  1. Age and gender:
    • RMS is most common in children younger than 10 years. However, it can affect teenagers and adults.
    • It is more prevalent in boys than in girls.
  2. Inheritance conditions: A few people are predisposed to developing forms of sarcomas due to mutations in their DNA acquired from their parents. A few rare hereditary conditions enhance the chance of RMS, such as:
    • Li-Fraumeni syndrome: This syndrome is found in family members who are predisposed to developing sarcomas, such as RMS and other cancers (breast cancer, leukemia).
    • Beckwith-Wiedemann syndrome: Children with this condition are more prone to develop sarcomas, such as RMS, but they are more likely to develop Wilm’s tumors (a form of kidney tumor) and other cancers.
    • Noonan syndrome: It is a syndrome in which children tend to be short, have cardiac problems, and develop physical and learning skills at a slower rate than other children. Children with this syndrome are at a higher risk of developing RMS.
    • Neurofibromatosis type I or Recklinghausen disease: It often generates many nerve tumors (particularly in cutaneous nerves), but it raises the likelihood of skeletal muscle sarcoma (RMS).
    • Costello syndrome: It is a rare occurrence in humans, yet children with this disease have high birth weights but later fail to develop normally and are short. They have disproportionately big heads. They are at risk of developing RMS and other malignancies.
  3. Exposure before birth:
    • Children who have been exposed to X-rays before birth may be at a higher risk of developing RMS.
    • Parental drug usage, such as marijuana and cocaine, has been proposed as a possible risk factor for RMS.

What are the 4 stages of rhabdomyosarcoma?

Cancer’s stage allows doctors to choose the most effective treatment. The four stages of rhabdomyosarcoma (RMS) include:

  1. Stage I RMS
    • Tumors in a favorable area, such as the orbit, head and neck area (except the parameningeal sites), and bile ducts.
    • These sarcomas can be of any size and have migrated to adjacent lymph nodes, but they have not metastasized (spread to distant sites).
  2. Stage II RMS
    • Tumors typically begin in undesirable locations, such as the bladder, prostate, legs, arm, parameningeal sites, or other regions of the body.
    • The tumor is less than 5 cm in diameter, and there is no sign of metastasis to the neighboring lymph nodes or distant portions of the body.
  3. Stage III RMS
    • Tumors typically begin in undesirable locations, such as the bladder, prostate, legs, arm, and other regions of the body.
    • The tumor is less than 5 cm in diameter but has spread to neighboring lymph nodes, or the tumor is greater than 5 cm in diameter and has spread to nearby lymph nodes.
    • However, cancer has not metastasized to other parts of the body in any circumstance.
  4. Stage IV rhabdomyosarcoma
    • Tumors can start anywhere in the body and vary in size. Stage IV RMS has spread to distant sections of the body, such as the lungs, liver, bones, or bone marrow, among other places.

What are the treatment options for rhabdomyosarcoma?

The treatment of rhabdomyosarcoma (RMS) depends on various factors, such as the type, stage, and grade of cancer. Surgery, chemotherapy, and radiotherapy are among the treatment options.

Chemotherapy

Chemotherapy is the use of anticancer medications to destroy cancer cells. It can be administered in the following cases:

  • To reduce the size of the tumor before surgery
  • Following surgery to lower the chance of recurrence of rhabdomyosarcoma

Medications are utilized and the duration of treatment is determined by the kind and stage of rhabdomyosarcoma.

Surgery

  • If it is possible, a child will undergo surgery to remove all or as much of the tumor as possible without injuring nearby tissue or organs.
  • The operation will be determined by the size of the tumor and its location in the child's body.
  • Chemotherapy is typically administered before surgery to reduce the tumor’s size and make it easier to remove surgically. If surgery is not an option, both chemotherapy and radiotherapy are used.

Based on the extent of the disease and how much it can be resected during the initial surgery, there are four clinical groups.

  1. Group I RMS
    • Localized and can be completely resected by surgery.
  2. Group II RMS
    • Localized RMS but in some cancer, cells have been found at the edges (margins) of the removed specimen. So, it is not completely resected by surgery.
    • It has spread to the nearby lymph nodes in the body.
  3. Group III RMS
    • Tumors that could not be removed completely and a few tumor cells are left, which could be seen with the naked eye.
    • The cancer cell may have spread too close to lymph nodes, but no sign indicates that it has spread (metastasize) to distant organs of the body.
  4. Group IV RMS
    • RMS has evidence of cancer spreading to other parts of the body, such as the lungs, liver, bones, bone marrow, or distant muscles or lymph nodes. So, it is not completely resected by surgery.

Radiotherapy

  • Radiotherapy uses high-energy rays to destroy cancer cells while causing as little injury to normal cells as feasible.
  • It may be administered after surgery at the site of the rhabdomyosarcoma.

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Clinical trials for rhabdomyosarcoma

  • Clinical research studies are available for almost every type of cancer and at every stage of the disease.
  • Clinical trials are intended to assess the efficacy of specific treatments. Trials are frequently designed to treat a certain stage of cancer, either as the initial kind of treatment offered or as a treatment alternative after other treatments have failed.
  • They can be used to evaluate drugs or therapies for cancer prevention, early detection, and side effect management.
  • Clinical trials are critical to advancing the understanding of this disease. Many interesting novel therapies are currently being explored. One should speak to the provider about taking part in clinical trials if they are eligible.

What are the treatment side effects of rhabdomyosarcoma?

  • Side effects of rhabdomyosarcoma treatment are common, and these will be reviewed before treatment begins.
  • The potential adverse effects vary depending on the type of treatment and the area of the body being treated. Side effects may include nausea and vomiting, hair loss, decreased resistance to infection, bruising and bleeding, exhaustion, and diarrhea.
  • These issues are transitory and can be mitigated with supportive treatment.

Survival rates in rhabdomyosarcoma vary depending on tumor size and location, as well as the quantity of tumor that can be removed. More than 70 percent of children with rhabdomyosarcoma that has not spread live a long and healthy life.

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Medically Reviewed on 4/20/2022
References
Image Source: iStock image

National Institutes of Health. Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version. https://www.cancer.gov/types/soft-tissue-sarcoma/patient/rhabdomyosarcoma-treatment-pdq#

American Cancer Society. What Is Rhabdomyosarcoma? https://www.cancer.org/cancer/rhabdomyosarcoma/about/what-is-rhabdomyosarcoma.html

Chen C, Dorado Garcia H, Scheer M, Henssen AG. Current and Future Treatment Strategies for Rhabdomyosarcoma. Front Oncol. 2019;9:1458. https://www.frontiersin.org/articles/10.3389/fonc.2019.01458/full

Cook Children's Health Care System. Rhabdomyosarcoma and Soft Tissue Sarcomas. https://www.cookchildrens.org/services/hematology-oncology/conditions/soft-tissue-sarcomas/

American Society of Clinical Oncology. Rhabdomyosarcoma - Childhood: Introduction. https://www.cancer.net/cancer-types/rhabdomyosarcoma-childhood/introduction