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.
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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.
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- CNS
- kidneys
- liver
- neck
- thorax
- pelvis
- back of the abdomen
If this tumor develops in the brain or spinal cord, it is
It typically affects children, particularly those under the age of
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
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
The SMARCB1 gene is responsible for suppressing the formation of tumors. Changes in its makeup may make tumor formation more likely.
In
Is it hereditary?
The
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
- 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
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
Additionally, the NCI says that most children with extra-cranial malignant rhabdoid tumors do not survive for more than a
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.
People may find financial assistance for treating cancers at the following organizations:
- CancerCare and their co-payment assistance foundation
American Cancer Society (ACS) - HealthWell Foundation
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
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
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.