Rhabdomyosarcoma (RMS) in Children

What Is Rhabdomyosarcoma (RMS)?

Rhabdomyosarcoma, pronounced (rab-doe-myo-sar-KO-muh) and often referred to as RMS or “Rhabdo,” is a cancerous tumor that develops in the body’s soft tissues. Rhabdomyosarcoma cells are fast-growing and can metastasize. RMS usually affects the muscles but can also affect the head, neck, bladder, vagina, arms, legs, trunk, or just about any body part.  

Kids can develop RMS at any age, often in children 2 and 6 years old and 15 and 19 years old. Boys tend to be affected slightly more often than girls.

What Causes Rhabdomyosarcoma (RMS) in Children?

The cause of RMS isn’t clear, but certain medical conditions can make some children more likely to develop it. These include: 

  • Li-Fraumeni syndrome:  a rare genetic disorder that increases the odds of developing cancer
  • Neurofibromatosis:  a condition that causes tumors to grow on nerve tissue
  • Beckwith-Wiedemann syndrome:  a congenital disease that causes too much growth in the body
  • Costello syndrome and Noonan syndrome:  both can cause deformities, and delays in development
  • DICER1 syndrome:  a genetic condition often discovered in embryonal RMS of the reproductive system

What Are the Signs and Symptoms of Rhabdomyosarcoma in Children?

Signs and symptoms of RMS depend on the tumor’s size and location. A mass or lump may appear on a child’s body and may be swollen but painless. Tumors may not be visible and reside within the body.

Rhabdomyosarcoma may cause headaches, bulging of an eye, or a lazy or drooping eyelid. RMS can affect urination and bowel movements, with blood in the urine or stool. If the tumor is pressing on a nerve, a child might feel tingling or weakness in the affected area.

How Is Rhabdomyosarcoma (RMS) Diagnosed?

If RMS is suspected, the doctor may order some additional tests such as:

  • Imaging: A CT scan, MRI, an X-ray, bone scan, PET scan, or ultrasound. These tests will help locate any tumors and determine if they have metastasized.
  • A Biopsy:  A tissue sample from a lump, mass, sore, or tissue for further analysis under a microscope. 
  • Blood tests: A complete blood count (CBC), liver panel, and blood chemistries provide information about how the body reacts to the tumor and any additional health concerns.
  • Bone marrow biopsy: The removal of a small amount of bone marrow tissue and examining it for cancer cells.

Types of Rhabdomyosarcomas

The two main types of RMS in kids are:

  1. Embryonal RMS: A tumor usually develops in the head and neck, genitals, or urinary tract and usually affects kids younger than six. In many cases, embryonal RMS responds well to intensive treatment.  
  2. Alveolar RMS: A tumor appears in teens and commonly affects the arms, legs, chest, or abdomen. It is a fast-growing tumor but more challenging to treat. Patients with alveolar RMS need intensive treatment.

How Is Rhabdomyosarcoma (RMS) Treated?

Staging treatment is a way to see if the disease has spread. This information helps the care team choose the best treatments.

Staging considers details like the size of the tumor (or tumors), how deeply the tumor has penetrated an organ, the area of the body where cancer began, and whether the tumor has spread to other organs. 

Other treatments may include:

  • Surgery:  To remove as much of the tumor as possible.
  • Radiation:  Treatment to destroy cancer cells and slow or stop them from spreading.
  • Chemotherapy:  Treatment designed to destroy cancerous cells of a tumor in a specific area of the body. 

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