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Symptoms, causes, treatment, and outlook

Rhabdoid tumors (RT) are large cell tumors that form in the soft tissues, such as the kidneys, liver, peripheral nerves, and the central nervous system (CNS). It is a rare, aggressive cancer that usually develops in young children and infants.

A 2018 article notes that although the outlook has improved for some individuals with rhabdoid tumors, the overall outlook remains negative.

This article covers what rhabdoid tumors are and their symptoms, causes, and treatment options.

A rhabdoid tumor is a rare, highly aggressive tumor that affects soft tissue anywhere in the body.

A 2022 article states that they often develop in the:

  • CNS
  • kidneys
  • liver
  • neck
  • thorax
  • pelvis
  • back of the abdomen

If this tumor develops in the brain or spinal cord, it is known as a subtype called an atypical teratoid rhabdoid tumor.

It typically affects children, particularly those under the age of 3 years and, most commonly, those between the ages of 11 and 18 months.

How common are they?

Rhabdoid tumors are rare. In the United States, there are fewer than 1,000 people with the disease. Some experts estimate that it affects fewer than 1 person for every 1 million people.

The National Institute of Health’s (NIH) Genetic and Rare Diseases Information Center notes that the age of onset for rhabdoid tumors can vary from before someone is born until they reach 11 years old.

The exact symptoms will depend on where the tumor initially develops. They typically start when the growing tumor puts pressure on surrounding tissue.

Symptoms may include:

  • abdominal pain
  • anemia
  • cerebral palsy
  • cranial nerve paralysis
  • headache
  • paralysis in the arm, leg, and in some cases, the face on one side of the body
  • high blood pressure
  • internal bleeding
  • irritability
  • swelling of the lymph nodes
  • nausea and vomiting
  • a reduced ability to control the movement of the eye
  • reduced appetite
  • respiratory issues
  • lesions on the skin that measure over 5 millimeters
  • weight loss
  • low platelet count

Researchers and experts are still unsure of the exact cause of rhabdoid tumors. However, according to the National Cancer Institute (NCI), nearly all these tumors are missing the SMARCB1 gene.

The SMARCB1 gene is responsible for suppressing the formation of tumors. Changes in its makeup may make tumor formation more likely.

In rare cases, it may develop due to changes to the SMARCA4 gene.

Is it hereditary?

The NCI states that malignant RTs do not run in families.

Due to its rarity, no standard treatment exists for rhabdoid tumors. However, doctors may try one or more different strategies to treat the tumor.

These may include:

  • surgery to remove as much of the tumor as possible
  • high dose chemotherapy to help kill a fast-growing tumor
  • radiation therapy to help shrink and kill the tumor

A doctor may also recommend a stem cell transplant or combining surgery with other treatment options. They may also recommend combining high dose chemotherapy with radiation therapy and stem cell transplant for optimal outcomes.

A parent or caregiver should discuss treatment options with a doctor. Each treatment comes with benefits and side effects to consider.

A child’s treatment team may vary according to their physical location and where the tumor developed in the body.

The team may consist of:

  • A primary care provider: These medical professionals can provide referrals to specialists and help coordinate overall medical care.
  • A pediatrician: A specialist in providing healthcare for children.
  • An oncologist: Specializes in diagnosing and treating cancer and malignant tumors.

Alongside the above medical professionals, the medical team may also include radiologists and surgeons.

The outlook for a rhabdoid tumor depends on:

  • the location of the tumor
  • whether it has spread
  • how much of the tumor remains following surgery

Typically, the outlook for this type of tumor is negative. The NCI says that children with atypical teratoid rhabdoid tumor, a CNS tumor, have a 5-year survival rate of 32.2%.

Additionally, the NCI says that most children with extra-cranial malignant rhabdoid tumors do not survive for more than a few years. However, those who receive a diagnosis after they reach 2 years old have a more positive diagnosis.

It is important to remember that due to the rarity of the disease, these survival rates may not be accurate. People should speak with the child’s care team to determine the chances of survival.

A doctor will likely first review the child’s symptoms. If they can find no other causes, they will likely order additional testing.

To diagnose rhabdoid tumors, a doctor will typically need imaging tests and a biopsy. Imaging tests may include the use of one or more of the following:

A biopsy involves taking a small sample of the tumor. The doctor will then send it to a lab to test for the presence of the SMARCB1 gene. Nearly every case of rhabdoid tumor has a mutation in this gene.

People may find financial assistance for treating cancers at the following organizations:

The ACS notes that childhood cancer is rare, meaning treatment can require travel. Some treatment centers are near a Ronald McDonald House, which provides low cost or free housing.

The ACS Hope Lodge program also offers families a free place to stay during the treatment.

To help with prescription medications, a person may wish to contact NeedyMeds or the PAN Foundation.

A person should also contact the billing departments at the hospitals. They may be able to work out payment plans, help find support for costs, and set someone up with a case manager who may be able to help find cost savings.

Children and their families may benefit from working with a mental health professional, such as a psychologist. A psychologist can help a family:

  • learn to cope with stress
  • process the emotions relating to cancer
  • help reduce distress during medical procedures
  • help families cope with grief and loss
  • support survivors
  • help manage sleep problems

The NCI and the ACS provide resources and information to help people cope with cancer.

Rhabdoid tumors are highly aggressive, rare soft tissue tumors that typically develop in young children and infants. It usually develops due to changes to the SMARCB1 gene.

Treatments for rhabdoid tumors can include surgery, chemotherapy, and radiation. A doctor may also recommend combining these therapies.

The outlook for RT is typically negative, but the survival rates may not be accurate due to the rarity of the disease. Parents and caregivers should speak with a doctor to determine the best course of treatment and likelihood of success.

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