Is Rhabdoid Tumor Curable?

Reviewed on 1/4/2022

What Is a Rhabdoid Tumor?

The prognosis for rhabdoid tumor is poor because they are very difficult to treat. Most children diagnosed with a rhabdoid tumor do not live more than a few years.
The prognosis for rhabdoid tumor is poor because they are very difficult to treat. Most children diagnosed with a rhabdoid tumor do not live more than a few years.

A rhabdoid tumor is a rare type of childhood tumor that often starts in the kidneys and other soft tissues such as the muscles attached to the bones of the skeleton.

These types of tumors are fast-growing and spread (metastasize) quickly to other parts of the body. 

Rhabdoid tumors that grow in the brain are called atypical teratoid rhabdoid tumors (ATRT). When these tumors grow outside the brain, they are called extra-cranial malignant rhabdoid tumors, malignant rhabdoid tumors, or MRTs. 

 

What Is the Life Expectancy for a Rhabdoid Tumor?

The average age at diagnosis is 15 months old and the prognosis is poor. In addition to growing and spreading rapidly, rhabdoid tumors are very difficult to treat. Most children diagnosed with a rhabdoid tumor do not live more than a few years. 

The prognosis depends on several factors, including the:

  • Size and location of the tumor
  • The extent of the disease
  • Tumor's characteristics 
  • Whether the tumor has spread (metastasized)
  • Tumor's response to treatment
  • Child’s age and overall health
  • Child's tolerance of medications, procedures, or therapies
  • Availability of new treatments

How Are Rhabdoid Tumors Treated?

Treatment for rhabdoid tumors usually involves a combination of one or more of the following: 

  • Surgery 
    • The first-line treatment in most cases
    • The doctor will attempt to remove the entire tumor
    • Depending on the size and location of the tumor, part of the kidney or all of the kidney may be removed
  • Chemotherapy 
    • Aggressive chemotherapy usually follows surgery
  • Radiation therapy 
    • May be given to children older than six months
  • Stem cell transplantation 
    • Treatment for malignant rhabdoid tumors may affect the body's ability to produce normal blood and immune cells so a stem cell transplant may be performed to help promote normal body and organ function 
  • Supportive care to prevent and treat:
    • Infections
    • Side effects of treatments
    • Complications

What Are Symptoms of Rhabdoid Tumor?

Symptoms of a malignant rhabdoid tumor include: 

What Causes Rhabdoid Tumor?

The exact cause of a malignant rhabdoid tumor is unknown. A gene called SMARCB1 (also called INI1, SNF5, and BAF47) is mutated in almost all rhabdoid tumors, including malignant rhabdoid tumors.

How Are Rhabdoid Tumors Diagnosed?

In addition to a medical and physical examination, diagnostic procedures for a malignant rhabdoid tumor are used to determine the type of tumor and whether the tumor has spread (metastasized). Rhabdoid tumors are definitively diagnosed by using histologic analysis.

Tests that may be helpful in the workup include:

  • Blood tests
    • Complete blood cell (CBC) count
    • Blood calcium
  • Urinalysis
  • Imaging studies
    • Abdominal computed tomography (CT) scan
    • Abdominal magnetic resonance imaging (MRI) can
    • Abdominal ultrasound
    • Chest CT scan 
    • MR, CT, fluorodeoxyglucose (FDG), and/or positron emission tomography (PET) imaging of the brain 
    • Bone scan (there is debate as to whether this is needed) 
  • Biopsy (tissue sample)
    • The sample is tested for mutations in a gene called SMARCB1 (also called INI1, SNF5, and BAF47), which is turned off in nearly all rhabdoid tumors
    • If a mutation is found, additional tests may be recommended

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Reviewed on 1/4/2022
References
Image Source: iStock Images

https://www.childrenshospital.org/conditions-and-treatments/conditions/m/malignant-rhabdoid-tumor

https://emedicine.medscape.com/article/993084-overview