Childhood rhabdomyosarcoma: 6 facts

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. This type of sarcoma grows in the muscles attached to the skeleton.

We spoke with medical oncologist Douglas Harrison, M.D., who treats pediatric cancer patients at MD Anderson Children’s Cancer Hospital, to learn more. 

What are common rhabdomyosarcoma symptoms?

Rhabdomyosarcoma symptoms vary depending on the cancer’s location. The most common symptom is a mass. Depending on where the mass appears on the body, symptoms may include: 

• Neck, chest, back, arm or leg: most commonly a lump on the skin or swelling that can be painful
• Eye area: bulging around the affected eye, which can lead to crossed eyes or blurry vision in advanced cases
• Ear or nasal sinus: earache, headache, nosebleeds or sinus congestion
• Bladder or prostate: painful urination, blood in the urine or vaginal bleeding
• Abdomen or pelvis: vomiting, belly pain or constipation
  

How is rhabdomyosarcoma diagnosed?

Rhabdomyosarcoma is normally confirmed through a needle or surgical biopsy which usually follows diagnostic imaging, such as MRI, CT scan or PET scan. A biopsy helps define the tumor’s subtype and in some cases can identify specific molecular changes in the tumor called a translocation. This enables the care team to make an accurate diagnosis.

The course of treatment typically depends on the specific subtype of rhabdomyosarcoma and whether the cancer has spread. 

What causes rhabdomyosarcoma?

In most cases, the cause of rhabdomyosarcoma is unknown. There are a few rare genetic conditions that children can inherit from their parents, which put them at increased risk for rhabdomyosarcoma. These include: 

• Neurofibromatosis type 1 (NF1)
• Li-Fraumeni syndrome 
• Dicer1 syndrome
• Costello syndrome 
• Beckwith-Wiedemann syndrome
• Noonan syndrome 

How is rhabdomyosarcoma typically treated? 
 
Treatment is usually determined based on the tumor size, location and whether it has spread. In children and adults, treatment for rhabdomyosarcoma usually involves surgery, along with chemotherapy in combination with radiation therapy. The number of treatments and type of chemotherapy used depend on whether the child has low-risk, intermediate-risk or high-risk rhabdomyosarcoma. The risk level is based on where the cancer starts, tumor size and metastatic spread. 

Radiation therapy shrinks a tumor by destroying any cancer cells that might remain after surgery and chemotherapy. Intensity modulated radiation therapy (IMRT), proton therapy, stereotactic body radiation therapy (SBRT) and internal radiation are most commonly used.  

Because of the physical properties of proton beams, proton therapy is ideal for pediatric cancer patients with tumors located near growing tissues, especially in the brain, spine, eyes, ears or mouth. This limits the possible damage to children whose bodies are still developing.

What should parents of a child with rhabdomyosarcoma know about choosing MD Anderson for treatment?  
 
Each sarcoma patient receives a comprehensive treatment plan tied into novel approaches for both adult and pediatric patients through our MD Anderson Children’s Cancer Hospital, Sarcoma and Orthopaedic Center and Proton Therapy Center.

What makes MD Anderson unique in sarcoma treatment is that our patients have access to more cutting-edge and early-phase clinical trials. Our experts are part of a joint adult/pediatric tumor board where they discuss treatments for each patient as a team with a comprehensive medical approach. 

Our patients have access to strong patient support programs and our fully accredited school designed to keep children on grade level. “We are able to deploy the best of adult cancer care with the best of pediatric cancer care in one space,” explains Harrison. “We stay in close contact with our patients and their families to ensure they get everything they need.”  

What are the latest advances in the diagnosis and treatment of rhabdomyosarcoma?

Clinical trials play an important role in making treatments even more effective. There are several worldwide clinical trials looking at patients with higher-risk rhabdomyosarcoma. There are also clinical trials looking at immunotherapy and targeted therapy for treating rhabdomyosarcoma patients. 
 
Harrison wants caregivers to know that current treatments offer a lot of hope for pediatric cancer patients and their families. “It takes a village, and our surgeons, radiation oncologists and pediatric oncology team work together to make sure our patients receive the best care,” he says. 
 
Request an appointment at MD Anderson online  or by calling 1-877-632-6789.